2019; 4(2): 1–44
Year 2019 Position Statement: Principles and Guidelines for Early
Hearing Detection and Intervention Programs
Table of Contents
Executive Summary.....................................
Background..................................................
Principles......................................................
Guidelines for Early Hearing Detection and
Intervention Programs..................................
Newborn Hearing Screening Programs.......
Pediatric Diagnostic Audiology.....................
Medical Evaluation.......................................
Early Intervention: Services for Infants/
Toddlers from Birth to Age Three Years and
Their Families..............................................
Medical Home and Ongoing Surveillance....
Protecting the Rights of Infants/Toddlers
and Families.................................................
EHDI Information Technology Infrastructure
Benchmarks and Quality Indicators.............
Future Directions..........................................
Research Needs..........................................
Acknowledgments........................................
References...................................................
The Joint Committee on Infant Hearing
Abbreviations
2 AAA – American Academy of Audiology
AABR – Automated Auditory Brainstem Response
AAP – American Academy of Pediatrics
3 ABA – American Board of Audiology
ABR – Auditory Brainstem Response
AHRQ – Agency for Healthcare Research and Quality
4 ANSI – American National Standards Institute
ASHA – American Speech-Language-Hearing Association
ASL – American Sign Language
4 ASSR – Automated Steady-State Response
BOA – Behavioral Observation Audiometry
CART – Communication Access Real-time Translation
5 CAEP – Cortical Auditory Evoked Potentials
CMV – Cytomegalovirus
cCMV – Congenital Cytomegalovirus
12 CDC – Centers for Disease Control and Prevention
CE – Click Evoked
17 CI – Cochlear Implant
dB – Decibel
DEC – Division of Early Childhood
DPOAE – Distortion Product Otoacoustic Emissions
DSL – Desired Sensation Level
ECMO – Extracorpeal Membrane Oxygenation
24 EHDI – Early Hearing Detection and Intervention
EHDI PALS – Early Hearing Detection & Intervention: Pediatric
28 Audiology Links to Services
HIPAA – Health Insurance Portability and Accountability Act
HL – Hearing Level
HRCT – High Resolution Computed Tomography
32 HRSA – Health Resources and Services Administration
IDEA – Individuals with Disabilities Education Act
IEP – Individualized Education Program
33 IFSP – Individual Family Service Plan
IOM – Institute of Medicine
34 ­ISO – International Organization for Standardization
JCAHO – Joint Commission on Accreditation of Health Care
Organizations
35 JCIH – Joint Committee on Infant Hearing
LTF/LTD – Loss-to-Follow-Up/Loss-to-Documentation
MRI – Magnetic Resonance Imaging
36 NCHAM – National Center for Hearing Assessment and
Management
37 NICHQ – National Institute for Children’s Health Quality
NIDCD – National Institute on Deafness and Other Communication
Disorders
37 NICU – Neonatal Intensive Care Unit
NIH – National Institutes of Health
NLM – U. S. National Library of Medicine
NQF – National Quality Forum
OAE – Otoacoustic Emissions
PCP – Primary Care Physician
PHI – Public Health Information
RECD – Real-Ear to Coupler Difference
SPL – Sound Pressure Level
TEOAE – Transient Evoked Otoacoustic Emissions
TJC – The Joint Commission
UNHS – Universal Newborn Hearing Screening
VRA – Visual Reinforcement Audiometry
WNL – within normal limits
The Journal of Early Hearing Detection and Intervention 2019; 4(2) 1
 Terminology
In this 2019 Statement, the Joint Committee on Infant Hearing (JCIH) seeks to use terms that: (a) are acceptable to a range of stakeholders, and
(b) clearly convey the intended meaning to the entire community. Because of the diversity of the committee’s composition and represented viewpoints,
a compromise resulted in choosing currently-recognized terms that reflect accepted, person-first language. In particular, the term infant or child who is
deaf or hard of hearing is intended to be inclusive of the entire spectrum of children, representing varied hearing levels. This spectrum includes children
who are deaf or hard of hearing whose hearing losses may be congenital or acquired, unilateral or bilateral, of any degree from minimal to profound,
and of any type, including conductive, sensory (sensorineural), auditory neuropathy, and mixed hearing condition, whether permanent, transient, or
intermittent. This spectrum includes those individuals who identify themselves as being a part of either, or both, the Deaf or hard-of-hearing
communities.

The commonly used term hearing loss is replaced, when grammatically appropriate to the written English language, with the terminology such as
hearing thresholds in the mild, moderate, severe, or profound range, acknowledging that for an infant who is born with hearing thresholds outside the
typical (normal) range, no loss has actually occurred. The JCIH recognizes that terms like hearing loss, hearing impairment, and hearing level have
different values or interpretations assigned to them depending on one’s cultural perspective. It is the intent of the JCIH to convey audiological concepts
using culturally-sensitive language whenever possible. However, there are times the term hearing loss is retained to clearly convey audiological
concepts/conditions, including references to late onset and progressive types. Further, use of the word normal as a type of hearing is replaced, when
appropriate, with the word typical to avoid any suggestion of the stigma of abnormality. Finally, in an effort to use clear language, the term refer for a
hearing screening result that is a not-pass outcome is avoided, due to lack of clarity and confusion about the meaning and implications of the word
refer. The term fail, which in years past had been discouraged in the belief that it would stigmatize infants, is recognized as a commonly-used term in
the medical world to describe the outcome of a binary screening and has been adopted for use in this document.

Executive Summary • Recognition of the value of implementation standards

Early Hearing Detection and Intervention (EHDI) activities
beginning at the birth hearing screening and culminating
in early intervention, have positively impacted outcomes
for children who are deaf or hard of hearing and their
families in the United States and world-wide. Universal
newborn hearing screening has resulted in significantly
lowering the average age of identification. Screening is a
necessary first step, but does not ensure the next critical
steps of timely identification and diagnosis of children who
are deaf or hard of hearing, amplification, and referral to
early intervention, all with the goal of promoting language
development.
The goal of EHDI is to assure that all infants are identified
as early as possible, and appropriate intervention initiated,
no later than 3–6 months of age. There is a body of
literature which demonstrates that children and families
experience optimal outcomes when these benchmarks are
met. Additionally, communication and linguistic
competence (in spoken language, signed language, or
both) are achievable when timelines are met, and when
optimal audiologic and early intervention services are
accessible. There remain critical areas of improvement
within the EHDI system to ensure newborns benefit from
early recognition and have access to appropriate supports.
This current 2019 document builds on prior Joint
Committee on Infant Hearing (JCIH) publications (2013
JCIH supplement on Early Intervention and 2007 JCIH
Guidelines), updating best practices through literature
reviews and expert consensus opinion on screening;
identification; and audiological, medical, and
educational management of infants and young children
and their families.
The current JCIH document includes the following
highlights.
Global Benchmarks and Rationale
• A review and reminder of the importance of early
diagnosis of hearing loss following best-practices.
for EHDI information systems.
• Recognition of the frequency, and impact, of
delayed-onset and/or progressive hearing loss in
infants and the need for continued surveillance of
auditory and speech-language development in all
infants, regardless of outcome of newborn hearing
screening.
• States who meet the 1-3-6 benchmark (screening
completed by 1 month, audiologic diagnosis by 3
months, enrollment in early intervention by 6 months)
should strive to meet a 1-2-3 month timeline.
Newborn Screening
• Endorsement of the necessity for audiology oversight of
hearing screening programs.
• Recognition of the critical need for the ability to
calibrate screening equipment using a uniform and
validated standard across all screening devices.
• Recognition of the need for manufacturers of screening
equipment to provide data on the proportion of children
who are deaf or hard of hearing who pass the
screening but are subsequently found to have a variety
of degrees and types of hearing loss.
• An endorsement, for well-born infants only, who are
screened by automated auditory brainstem response
(AABR) and do not pass, that rescreening and passing
by otoacoustic emissions testing is acceptable, given
the very low incidence of auditory neuropathy in this
population.
• An endorsement of rescreening in the medical home in
some circumstances. If the rescreening is performed in
the provider’s office, the provider is responsible for
reporting results to the state EHDI program.
Diagnostic Audiology and Audiological Interventions
• A review of current research on the physiologic/
electrophysiologic methods for diagnostic audiologic
evaluation of hearing in infants.
• A reaffirmation of the importance of fitting hearing aid
amplification using objective, evidence-based protocols
to ensure maximal audibility.

The Journal of Early Hearing Detection and Intervention 2019; 4(2) 2

Early Intervention and Family Support
• Reaffirmation of the need to provide families with
individualized support and information specific to
language and communication development to
support children who are deaf or hard of hearing by
providing exposure to language models at the earliest
possible age to ensure optimal cognitive, emotional,
and educational development.
• Recognition that some families may benefit from infant
mental health supports. Infant mental health is a field
of research and practice that focuses on optimizing
social, emotional, behavioral, and cognitive
development of infants in the context of the emerging
relationships between parents and infants.
Medical Considerations
• Reaffirmation of the need for otologic/medical
evaluation and management of the newly-identified
infant to be carried out as soon as possible following
confirmation, in an effort to address potentially
reversible conditions, discover associated medical
disorders that can impact the infant’s general health,
and identify conditions that can impact communication
strategy choice.
• Recognition that Congenital Cytomegalovirus has a
larger impact than previously recognized.
• Updated risk indicators for congenital hearing
conditions, including a new table with specified
intervals for audiologic evaluation.
• Consideration of reduction in the FDA-approved age for
cochlear implantation to less than 12 months.
JCIH’s guiding principle is for continued improvements in
the EHDI system. This includes lowering the age of
identification and diagnosis of infants, as well as ensuring
timely and effective interventions to improve language and
social-emotional outcomes in children who are deaf or
hard of hearing. Amplification (hearing aids,
cochlear implants, bone conduction aids) and early
language interventions (whether signed language, spoken
language or both) should be based on best practice
protocols and evidence-based practice as soon as
possible following a diagnosis of hearing loss.
The Joint Committee on Infant Hearing (JCIH)
endorses early detection and early intervention for all
infants who are, or who are at risk of being or becoming,
deaf or hard of hearing. The goals of early hearing
detection and intervention (EHDI) are to maximize
language and communication competence, literacy
development, and psychosocial well-being for children who
are deaf or hard of hearing. Without appropriate language
exposure and access, these children will fall behind their
hearing peers in communication, language, speech,
cognition, reading, and social-emotional development, and
delays may continue to affect the child’s life into adulthood.
With early detection and appropriate, targeted
intervention, developmental milestones for an infant who
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
is deaf or hard of hearing can be expected to be achieved,
more accurately reflecting the child’s true potential
(Tomblin, Oleson, Ambrose, Walker, & Moeller, 2014;
Yoshinaga-Itano, Baca, & Sedey, 2010). Focusing on the
importance of prompt diagnosis and timely, high-quality
early intervention for such infants, EHDI systems should
facilitate seamless transitions for infants and their families
through the processes of screening, audiologic and
medical diagnosis, and intervention.
Background
In the absence of early recognition (and with the resulting
lack of access to language), a child who is deaf or hard of
hearing in infancy, can experience delays in speech and
language development, academic achievement, and social
and emotional outcomes. Historically, children who are
deaf or hard of hearing were not identified until
two-to-three years of age, and those with hearing
thresholds between 25 and 40 dB hearing level (HL) were
often undetected until school age (Yoshinaga-Itano, Sedey,
Coulter, & Mehl, 1998).

In the late 1980s, federal agencies such as the United
States (U.S.) Health Resources and Services
Administration (HRSA) and the National Institute on
Deafness and Other Communication Disorders (NIDCD)/
National Institutes of Health (NIH) recognized the new and
evolving technologies for screening hearing in newborns,
and appreciated the potential of universal newborn hearing
screening (UNHS) to allow early diagnosis and prompt
intervention for infants who were born deaf or hard of
hearing (NIH, 1993). Soon thereafter in the 1990s,
newborn hearing screening programs became a reality in a
number of states. In the new millennium, newborn
hearing screening has become the standard of care, not
only for U.S. hospitals and birthing centers, but also for
many countries around the world (Olusanya, 2011; van
Dyk, Swanepoel, & Hall, 2015).
EHDI programs throughout the nation have demonstrated
not only the feasibility of UNHS programs, but also the
increasingly measurable benefits of early identification and
intervention (Ambrose, Unflat Berry, et al., 2014; Tomblin
et al., 2014; Tomblin et al., 2015). Despite on-going
challenges to track all infants who do not pass initial
hearing screening, and despite the resulting delays in
diagnosis and shortcomings in assuring adequate
follow-up, both the average age of diagnosis and the
average age of referral to early intervention have steadily
decreased (Holte et al., 2012). As a result, each year in the
United States some 5,000 infants who are deaf or hard of
hearing and their families have the opportunity to
experience improved outcomes in the child’s language
development (through early access to spoken and/or
signed language), as well as improved outcomes in the
child’s overall well-being (Williams, Alam, & Gaffney,
2015).
3
 Principles 11. Professionals with appropriate training should
provide ongoing surveillance of communication
All identified and at-risk children and their families should development to all children with or without risk
have access to resources necessary to reach their indicators.
maximum potential. The following principles provide the 12. Appropriate interdisciplinary early intervention
foundation for effective EHDI systems and have been programs for identified infants and their families
updated, building on and expanding the practices should be provided by professionals knowledgeable
stipulated and endorsed in the JCIH 2007 position about the needs and requirements of children who
statement. are deaf or hard of hearing (JCIH, 2013).
13. Early intervention programs should recognize
EHDI 1-3-6 Goals evidence-based practices and build on strengths,
1. All infants should undergo hearing screening prior to informed choices, language traditions, and cultural
discharge from the birth hospital and no later than beliefs of families they serve.
one month of age, using physiologic measures with 14. EHDI information systems should be designed and
objective determination of outcome. implemented to interface with clinical electronic health
2. All infants whose initial birth-screen and any records and population-based information systems to
subsequent rescreening warrant additional testing allow the exchange of electronic health information for
should have appropriate audiologic evaluation to the purposes of outcome measurement, quality
confirm the infant’s hearing status no later than 3 improvement, and reporting the effectiveness of EHDI
months of age. services for the patient/family within the medical
3. A concurrent or immediate comprehensive otologic home, healthcare community, state, and federal
evaluation should occur for infants who are confirmed levels.
to be deaf or hard of hearing.
4. All infants who are deaf or hard of hearing in one or Guidelines for Early Hearing Detection and
both ears should be referred immediately to early
Intervention Programs
intervention in order to receive targeted and
appropriate services.
The guidelines presented in this 2019 position statement
5. A simplified, coordinated point of entry into an
were developed to update the 2007 JCIH position
intervention system appropriate for identified children
statement and serve to support the goals of universal
is optimal.
access to hearing screening, evaluation, and intervention
6. Early intervention services should be offered through
for newborns and infants. The guidelines provide current
an approach that reflects the family’s preferences and
information on the development and implementation of
goals for their child, and should begin as soon as
successful EHDI systems. Studies have demonstrated that
possible after diagnosis but no later than six months
current screening technologies are effective in
of age and require a signed Part C of IDEA
identifying hearing thresholds of approximately 35–40 dB
(Individuals with Disabilities Education Act, 2004)
HL and greater (Norton et al., 2000a); however, mildly
Individualized Family Service Plan.
elevated hearing thresholds are not identified using current
7. The child and family should have immediate access,
screening technologies and even mildly elevated hearing
through their audiologist, to high-quality, well-fitted,
thresholds can impact speech and language development
and optimized hearing aid technology. Access should
(Walker et al., 2015). Language, speech, social-emotional,
also be assured, depending on the child’s needs, to
and academic development may be affected when
cochlear implants (CI), hearing assistive technologies,
children who are deaf or hard of hearing lack full or
and visual alerting and informational devices.
complete access to linguistic input (Dillon, Cowan, &
Ching, 2013; Robertson, Howarth, Bork, & Dinu, 2009;
Updated Principles Beyond EHDI 1-3-6
Yoshinaga-Itano, et al., 1998). Specific attention is given in
8. The EHDI system should be family-centered with
this document to infants who receive care in the neonatal
infant and family rights and privacy guaranteed
intensive care unit (NICU) because research data indicate
through informed and shared decision-making, and
that this population is at higher risk for hearing loss
family consent in accordance with state and federal
(Moeller, Carr, Seaver, Stredler-Brown, & Holzinger, 2013).
guidelines.
9. Families should have access to information about all
Regardless of previous hearing-screening outcomes, all
resources and programs for intervention, and support
infants with or without risk factors should receive ongoing
and counseling regarding the child’s educational and
surveillance of communicative development beginning at 2
communication/language needs.
months of age during well-child visits in the medical home
10. All infants and children, regardless of newborn
(AAP Committee, 2017). This recommendation provides
hearing screening outcome, should be monitored
an alternative, more inclusive strategy of surveillance of all
within the medical home according to the periodicity
children within the medical home based on the pediatric
tables regarding their communication development
periodicity schedule (AAP Committee, 2017). All infants
(American Academy of Pediatrics [AAP] Committee,
who do not pass the speech-language portion of a
2017).
developmental screening in the medical home or for whom

The Journal of Early Hearing Detection and Intervention 2019; 4(2) 4

there is a concern regarding hearing or language should
be referred for speech-language evaluation and audiology
assessment. This protocol permits the detection of
children with either missed neonatal or delayed-onset
hearing loss, irrespective of the presence or absence of a
high-risk indicator.
Depending on the screening technology selected, infants
with auditory neuropathy may not be detected through a
UNHS program. Given the low incidence of auditory
neuropathy in the well-baby nursery, the JCIH
recommends the use of either automated auditory
brainstem response (AABR), or otoacoustic emissions
(OAEs), or both for initial screenings and/or rescreening.
However, the JCIH continues to recommend AABR
screening and rescreening protocols in the NICU to allow
for detection of auditory neuropathy.
Optimal EHDI programs have been defined as meeting
the EHDI 1-3-6 goals. To provide appropriate access to
language stimulation and intervention services as soon as
possible, EHDI programs meeting current targets might
consider setting a new target of 1-2-3 months (screening
completed by one month of age, audiologic diagnosis
completed by two months of age, and early intervention
initiated no later than three months of age). The
earliest possible age of identification is encouraged for two
reasons. First, the infant can receive earlier intervention for
auditory and/or visual access to language. Second,
objective audiologic testing can be completed without
sedation during the natural sleep that occurs when
newborn/infants are young enough to sleep for prolonged
periods of time. It may not be appropriate to apply this
timeline to infants receiving care in the NICU. Because the
majority of very preterm infants may still be in the NICU at
3 months of age, a recommendation is made that for very
preterm infants with prolonged hospitalization, a diagnostic
audiologic evaluation prior to discharge from the NICU be
completed. Infants identified as being deaf or hard of
hearing could then be referred directly for early
intervention and audiological follow-up services at the time
of discharge.
Primary care physicians need to be aware of the necessity
for monitoring communication and language skills
according to the most recent developmental periodicity
tables (AAP Committee, 2017). Any child who
demonstrates delayed auditory and/or communication
skills development, even if that child passed newborn
hearing screening, should be promptly referred for
audiologic evaluation. Additionally, any child whose
parents/caregivers express concern regarding auditory
and/or communication skills development, even if that child
passed newborn hearing screening, should be promptly
referred for audiologic evaluation.
Newborn Hearing Screening Programs
Multidisciplinary teams of professionals including
audiologists, physicians, and nursing personnel are
needed to establish the UNHS component of EHDI
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
programs (JCIH, 2013; Moeller et al., 2013). An audiologist
with experience in evaluating newborns and young
children should be involved in the development and
oversight of each component of the hearing screening
program, particularly at the level of statewide
implementation and, whenever possible, at the individual
hospital level. Hospitals and agencies should also
designate a physician/provider to oversee the medical
aspects of the EHDI program.
Each team of professionals responsible for the
hospital-based UNHS program should review the hospital
infrastructure in relationship to the screening program.
Evidence-based research is needed to support the
reliability and validity of all screening equipment used,
whether OAE or AABR. Hospital-based programs must
consider a variety of issues, including the population of
infants to be screened, screening technology choices,
validity of the specific screening device, screening
protocols (including the timing of screening relative to
hospital discharge), availability of qualified screening
personnel, suitability of the acoustical and electrical
environments, follow-up referral criteria and pathways,
information management, and quality control and
improvement. Reporting and communication protocols
must be well-defined, and such protocols must include the
content of reports to physicians and to families,
documentation of results in medical records, flow of
screening result information from hospital-based medical
records to outpatient records, and methods for reporting
to state registries and national data sets. Hospitals should
identify and define the referral pathway and systems for
ensuring that each baby who does not pass in-patient
screening has access to a pediatric audiologist or
certified/registered screener who can complete the
outpatient rescreening, and when indicated, refer for or
complete a diagnostic audiologic evaluation according to
the EHDI 1-3-6 or 1-2-3 goals.
Audiology Oversight of Newborn Hearing Screening
Programs
Audiology oversight is recommended for all state/territory
hearing screening programs, both at the systems level and
at the individual program level. The systems-
oversight audiologist (in collaboration with medical and
nursing personnel) should design and implement
procedures to (a) conduct periodic on-site and/or remote
surveillance (e.g., phone, email, and web-conference) of
the individual hospital programs, (b) provide oversight and
participate in the writing of the policies and procedures for
screening, (c) train staff and assure competency, and (d)
monitor program statistics and quality assurance. Referral
pathways and timelines should be developed in
conjunction with community resources and the state EHDI
program to ensure timely rescreening of those infants who
do not pass their birth hearing screens, as well as timely
and appropriate diagnostic audiologic evaluations.
Across the fifty states and nine territories, different
models exist for audiology oversight of an individual UNHS
program. In some state EHDI systems, individual hospital
5
programs contract with an outside screening entity, an
audiologist, or audiology program, while others provide this
oversight from within their own staff audiology personnel.
Some systems provide oversight over a specific
geographic region. An audiologist with requisite knowledge
should oversee all aspects of each individual UNHS
program. This should entail, at a minimum:
1. Selection of screening technology and equipment
based on the population(s) to be screened.
• Consideration of whether the equipment will be used
for well babies, infants receiving care in the NICU,
or both (e.g., due to the maturation of the auditory
neural pathway, pre-term infants and/or older
infants may not meet the criteria for automated
screening protocols whose pass/fail outcome is
based on average responses from a term baby).
• Comprehension of the manufacturer’s required
age-limits for the specific screening equipment
selected to conduct valid screening of premature,
as well as older infants.
• Referral of an infant who is too old for screening
using automated equipment according to the
manufacturer’s validation statement to a diagnostic
audiologic evaluation if appropriate (e.g., if the
infant is in the NICU, the diagnostic evaluation can
be conducted as an in-patient procedure).
2. Confirmation that initial equipment calibration
performed by the manufacturer or distributor is done in
a manner consistent with purported screening
parameters.
• Establishment of a method by which an
independent entity (e.g., hospital clinical
engineering, local special instrument distributors
who conduct routine annual audiometric calibration
of all equipment, etc.) can perform calibration or
provide oversight to ensure that equipment
parameters remain stable and appropriate.
• Calibration of test stimuli performed in a coupler
appropriate to the transducer and in reference to a
published calibration standard (American National
Standards Institute [ANSI; http://www.ansi.org], or
International Organization for Standardization [ISO;
http://www.iso.org/iso/home/standards.htm]).
• Calibration procedures should be fully transparent to
the end-users, including such metrics as stimulus
parameters (i.e., intensity and bandwidth of the
stimulus, relationship of stimulus metrics to hearing
threshold estimates as a binary outcome of pass/
fail, and expected pass/fail metrics for both the
well-baby and NICU populations).
• Requirements for troubleshooting, annual
calibration, and expected performance in the
population to be screened should be reviewed by
the audiologist.
3. Development and implementation of protocols for
training and certifying competence of new screeners.
• Regular educational in-service training for all
program personnel should be an ongoing
commitment to assess and ensure continued
competence.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
• The audiologist, along with the manufacturer, should
provide training to the screening personnel in the
correct use of the screening equipment.
• The audiologist should provide training to screeners
in optimizing infant state for screening. This
includes ensuring a quiet, preferably sleeping baby,
and using techniques to verify that the ear canal is
open and patent to the extent possible before the
earphone or probe is placed.
• The audiologist may, depending on the size and
needs of the program, conduct a train-the-trainer
educational program to produce an adequate and
continuing supply of screeners.
4. Development and implementation of policies,
procedures, and protocols, in conjunction with nursing,
medical, and other personnel (e.g., care coordination),
based on best-practices and in accordance with state
regulations.
• Identify the screening methods, timing, and
equipment to be used to conduct the screening,
including provisions for readily available backup
equipment in the event of an equipment malfunction.
• Provide samples or scripts of information (spoken,
signed, and/or written in the language of the home)
given to the family regarding the screening outcome,
and including: (a) a definition of the term pass and
a definition for the word fail (or did not pass, or refer,
alternate terms that are sometimes used for a
non-passing result); (b) the necessity for appropriate
follow-up rescreening when indicated; (c) the
importance of early and timely completion of such
rescreening and diagnostic audiologic evaluations,
if indicated; (d) the benefits of early intervention for
the infant who is subsequently identified as being
deaf or hard of hearing, and (e) a clear statement
regarding next steps. When appropriate,
information regarding third-party coverage of the
screening and audiologic diagnosis costs should be
provided. Identify the procedures for communicating
to the family that the infant requires additional
follow-up testing due to the results of the screening
or missed screening. Communication should occur
in a manner that is culturally and linguistically
appropriate, and should be provided orally for
hearing families (with a foreign language interpreter
as indicated), as well as in writing or through
audio-visual means. Families whose primary
language is American Sign Language (ASL) or
another sign language, or a language different from
English should be provided with an interpreter.
• Provide hearing screening results and outcomes to
the infant’s medical home or primary care provider
and to the state EHDI program.
• Both the family and the primary-care provider should
be advised that passing a hearing screening
performed either by OAE or by AABR testing does
not imply that hearing thresholds are within normal
limits (WNL), only that thresholds are not greater
than approximately 35-40 dB HL.
6
 • Clearly communicate that a pass outcome implies
that the infant passes both ears simultaneously (in
the same screening session). Specifically, an infant
who does not pass both ears in the same screening
session, even if each ear has separately passed a
screening, does not constitute a pass outcome.
Parents should not be advised, “one ear passed and
the other ear did not pass.”
• Describe the training and supervision of individuals
with responsibility to inform the family of screening
results, ensuring that the family understands the
importance of immediate follow-up when the baby
does not pass the newborn hearing screening.
• Provide the family with an appointment for
outpatient rescreening at the time of discharge from
the birth hospital as best practice to decrease the
likelihood of loss-to-follow-up/loss-to-documentation
(LTF/LTD).
5. Development and implementation of quality assurance
procedures that include monitoring screening
statistics.
• Outline the procedures for documenting and
transmitting the results of the screening in a
manner consistent with the Health Insurance
Portability and Accountability Act (HIPAA) and other
compliance requirements (see the section titled
“Protecting the Rights of Infants/Toddlers and
Families” below for an expanded discussion).
• Identify and document written procedures to ensure
that an infant who does not pass the hearing
screening while still in the hospital (or who never
completes hearing screening while at the hospital)
will receive a timely initial or follow-up screening as
an outpatient.
• Identify the procedures for reporting data on a
regular basis as required by the state EHDI
program. Data reporting should be accomplished
using a secure, web-based data system, with
individual infant data being reported by the hospital
directly to the state EHDI system.
6. Specification of program staffing requirements and
definition of the responsibilities of each staff member:
screening nurses and other screening personnel,
audiology director, medical director.
7. Development of standard operating procedures that
outline processes and steps to follow when reviewing
and recording hearing screening status in the
discharge plan or transfer plan for all newborn infants.
Safeguards should be in place to ensure that infants
who are transferred to a different unit or facility and
who develop a new risk factor are re-screened prior to
discharge.
8. Ensure acceptable, independent, on-site oversight by
an audiologist who is either employed by the
hospital, or is otherwise independent of the contracted
entity in screening programs where services are
contracted through an outside entity.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Objective Physiologic Screening Measures
Objectively-determined physiologic measures must be
used to screen newborns and young infants to identify
those who may be deaf or hard of hearing (Cone-
Wesson et al., 2000; Johnson et al., 2005a; Norton et al.,
2000b). Currently such measures include
automated OAE and auditory brainstem response (ABR).
Automated OAE protocols use either transient-evoked
OAEs (TEOAE) or distortion-product OAEs (DPOAE). Both
automated OAE and ABR technologies provide
noninvasive recordings of physiologic activity underlying
normal auditory function. Both are easily performed in
neonates and infants, and both have been successfully
used for UNHS programs (Gravel et al., 2000; Norton et
al., 2000b). There are, however, important differences
between the two measures. OAE measures a physiologic
response from the cochlear outer hair cells, while ABR
measurements reflect both cochlear status, as well as
auditory neural function extending beyond the cochlea into
the brainstem. Thus, the ABR response reflects
activity from a greater portion of the auditory pathway than
does the OAE. For this reason, ABR will result in a failed
outcome when screening infants with auditory neuropathy,
whereas screening with OAEs will result in a pass for the
baby with auditory neuropathy.
Both OAE and Auditory Brainstem Response (ABR)
screening technologies can be used to detect whether
the child has a sensory (cochlear) hearing loss/condition
(Norton et al., 2000a). However, the results obtained using
either technology are affected by concomitant outer ear or
middle ear dysfunction that might be present.
Consequently, conditions of the outer ear and/or middle
ear may result in a failed hearing screening in the
presence of normal cochlear and/or neural function. Use of
OAE as a screening tool is likely to result in a
higher fail rate in the immediate post-birth period as
compared with AABR (van Dyk et al., 2015).
Some infants who pass newborn hearing screening will
later be identified as deaf or hard of hearing (Johnson et
al., 2005b). Although this later-detected hearing loss may
reflect delayed-onset or progressive changes in hearing,
both AABR and OAE screening technologies will fail to
identify children who are deaf or hard of hearing with
hearing thresholds that are considered borderline or mild
or are in an isolated frequency-region (Young, Reilly, &
Burke, 2011). The hearing threshold detected with AABR
screening technology is slightly higher (40 to 45 dB HL) as
compared with OAE technology (30 or 35 dB HL).
Therefore, there is a slightly greater chance of hearing
thresholds between 25 and 40 dB HL going
undetected when the initial screening is performed using
AABR. Forty-two percent of children who failed a TEOAE
screen but did pass an AABR screen were subsequently
found to have hearing thresholds greater than 45 dB HL
(Levit, Himmelfarb, & Dollberg, 2015). Conversely, OAE
technology failed to detect auditory neuropathy of any
degree (Johnson, 2005a).
7
Interpretive Criteria
Each hearing screen comprises simultaneous assessment
of both the right and left ears. Criteria for hearing
screening outcomes should reflect an optimal balance
between sensitivity and specificity, considering the
prevalence of infant hearing loss (Gorga et al., 2006),
manufacturer-reported test performance, and the goal of
identifying elevated hearing thresholds that can affect
spoken language development. Screening technologies
that incorporate automated response detection algorithms
were developed to eliminate the need for individual test
interpretation, reduce the effects of screener bias or
operator error on test outcome, and ensure screening
consistency across infants, test conditions, and screening
personnel. When statistical probability is used to make
pass/fail decisions, as is the case for OAE and AABR
screening devices, the likelihood of obtaining a pass
outcome by chance alone is increased when screening
is performed repeatedly (Type I error). The increased
probability of a Type I error must be incorporated into the
policies of rescreening, thus JCIH has renewed the
recommendation that (a) no more than two high-quality
hospital-based screenings should be performed prior to
hospital discharge, and (b) only one high-quality
outpatient rescreen be performed prior to referral to a
pediatric audiologist for the child who needs follow-up
testing after the outpatient rescreen. A high-quality screen
implies that the infant is sleeping or resting quietly
without movement throughout the screening period, and
that patency of the ear canal is assured to the extent
possible prior to initiating the screen.
Calibration of Hearing Screening Equipment
Both screening and diagnostic audiologic measures that
are based on electrophysiologic responses employ
stimuli for which there is presently no universally-accepted
calibration standard. Most current screening technologies
use brief-duration stimuli that lack calibration standards set
by the American National Standards Institute (ANSI; http://
www.ansi.org). The ISO has a standard that specifies ref-
erence threshold sound pressure levels (SPL) and enve-
lopes of standard tonebursts and clicks for test signals of
short duration applicable to the calibration of audiometric
equipment (http://www.iso.org/iso/home/standards.htm).
Calibration of transient stimuli used in infant hearing
screening programs is complicated by variability in
stimulus parameters (e.g., stimulus, rate, duration) and by
types of transducers used in automated devices. Differing
transducers and the use of different ear couplers lead to
variability in both the stimulus level and spectrum. This is
complicated by the fact that there are no universally
accepted conversion factors for transforming adult
threshold values to infant/pediatric use. Furthermore, while
there is general agreement regarding the relationship of
electrophysiological threshold measures to subsequent
behavioral thresholds within group data, accuracy in
predicting behavioral thresholds varies for the
individual child (Gorga et al., 2006; McCreery, Kaminski, et
al., 2015). In the absence of universally accepted
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
specifications of stimulus level and spectrum for use in
newborn hearing screening and infant hearing testing, it is
imperative that manufacturers of screening equipment
provide objective and transparent calibration information,
as well as the pass/fail rates in populations of newborns
and infants demonstrated to have normal hearing and
those confirmed as deaf or hard of hearing. Even within a
single manufacturer, it has been reported that calibration
intensities and spectra differed between two devices
designed to screen infant hearing, with a significant
difference in referral rate that was attributed to these
dissimilarities (Hofmann, Luts, Poelmans, & Wouters,
2012). JCIH called for a calibration standard for newborn
screening using ABR in 2007, however, in the United
States, there is not yet an ANSI standard.
Finally, it is suggested that, until such time as an ANSI
standard is developed, practicable, and promulgated,
manufacturers should provide calibration information per
the ISO standard that is available in countries outside the
United States. The question of false-negative test results
(i.e., pass newborn hearing screening when hearing
thresholds are elevated) continues to deserve scrutiny.
Although it is not the goal of universal newborn hearing
screening to identify all infants who are deaf or hard of
hearing (e.g., infants with auditory neuropathy will be
missed when screened by OAE; infants with minimal-mild
hearing thresholds will be missed when either technology
is used), the importance of acknowledging, and estimating
the occurrence of false-negative outcomes must continue
to receive attention. This is particularly critical given the
increasingly-recognized significance of even mild threshold
elevation in infants and young children (Walker et al.,
2015).
Timing of Newborn Hearing Screening
Infants should have their hearing screened as close to
discharge as practicable, while at the same time allowing
sufficient time for a single repeat screen to be performed
if the infant does not pass the first screen. The second
screen, should it be required, should not be performed
immediately following the first screen, but should occur
at least several hours later. Infants with congenital aural
atresia in one or both ears or with visible pinna/ear canal
deformity such as stenosis or severe malformation should
not be screened in either ear but should be referred for
diagnostic audiologic evaluation immediately upon
discharge. The diagnostic audiologic evaluation can also
be accomplished while the infant is in the NICU or other
inpatient hospital unit.
For some infants in the NICU (e.g., infants on ventilators),
it may not be feasible or practical to complete a hearing
screening prior to one month of age due to the high
likelihood of middle ear effusion, noise interference, and
electrical interference from equipment. Alternative
arrangements should be made for completing the hearing
screening on these infants at a time when they are
medically stable.
8
Screening Protocols in the Well-baby Nursery
Many inpatient well-baby screening protocols provide one
hearing screening and, when necessary, a repeat
screening prior to discharge from the hospital, using the
same technology with both screenings. Use of either
AABR or OAE technology will detect hearing thresholds of
children who are deaf or hard of hearing with
peripheral conductive and sensorineural etiology, within
the limits of the testing threshold of the selected
technology (Norton et al., 2000b). When AABR is used as
the single screening technology, neural auditory disorders
(auditory neuropathy) can also be detected. Some
programs use a combination of screening technologies for
the well-baby nursery (OAE testing for the initial screening,
followed by AABR for rescreening), a so-called two-stage
protocol (Lin, Shu, Lee, Lin, & Lin, 2007). Such a protocol
can minimize initial disposable costs and decrease the fail
rate at hospital discharge, thereby reducing the
subsequent need for outpatient follow-up. Using this
approach, infants who fail an OAE screening but
subsequently pass an AABR are considered a screening
pass.
In the previous 2007 JCIH Statement, it was
recommended that infants in the well-baby nursery who
do not pass an AABR screening not be rescreened and
passed using OAE technology because such infants are
presumed to be at risk of having a subsequent diagnosis of
auditory neuropathy. At the same time, there is a very low
incidence of auditory neuropathy in the well-baby nursery
(6–30/100,000 births; Korver, van Zanten,
Meuwese-Jongejeugd, van Straaten, & Oudesluys-Murphy,
2012). Korver and colleagues note that there is not much
evidence available upon which to draw a definitive statistic,
and that the evidence that is available is variable. There
continues to be an unacceptably high loss-to-follow-up
statistic for infants who fail the birth screen and then fail
to return for rescreening as an outpatient (Korver et al.,
2012). Given these considerations, a new
recommendation is made that screening in the well-baby
nursery may be accomplished using either OAE or AABR,
with the second (re-) screen (second in-hospital screen),
conducted using either technology. The recommendation
to rescreen using only AABR technology for the infant who
fails initial screening performed with AABR
technology continues to be the Committee’s preferred
protocol. Less than 1% of newborns in the well-baby
nursery had an AABR fail with an OAE pass as
inpatients, and none exhibited this pattern as outpatients
(Berg, Prieve, Serpanos, & Wheaton, 2011). Specifically,
re-screening with OAE after failing an AABR is acceptable
with the caveat that a baby with auditory neuropathy in
the well-baby nursery will be missed using this protocol.
In essence, the JCIH asserts that any pass-screen result
of both ears in the same screening session using either
technology prior to hospital discharge is acceptable as a
passed hearing screen (see “Rescreening in the
Outpatient Setting” below for an expanded discussion).
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Screening Protocols in the Neonatal Intensive
Care Unit
Infants who have received care in the NICU represent 10%
to 15% of the newborn population and have been shown
to have a higher prevalence of elevated hearing thresholds
compared to infants from well-baby nurseries (Robertson
et al., 2009; Vohr et al., 2000).
Not only is there a higher prevalence of hearing loss in this
population, there is also a higher risk of auditory
neuropathy in infants with a history of hyperbilirubinemia
and those administered a mycin-class antibiotic (Berg
et al., 2005). For this reason, the sole use of AABR for
hearing screening is recommended for infants who have
received care in the NICU. In addition, infants cared for in
the NICU who do not pass the AABR should be
(a) referred directly to an audiologist for rescreening, and
(b) if indicated, given a comprehensive audiological
evaluation including diagnostic ABR.
There is no definitive information regarding the intensity of
the screening level for AABR equipment. It is known that
some infants who pass ABR screening do not pass OAE
screening, and that some infants who pass ABR
screening subsequently are found to have elevated
hearing thresholds. Whether these losses were present at
birth or developed later in infancy is not known. There is
evidence suggesting that OAE screening is more sensitive
to mild hearing thresholds than AABR. In a recent study by
Levit and colleagues (2015), 42% of the infants who failed
OAE screening, passed the AABR screening and were
subsequently found to have hearing thresholds greater
than 45 dB HL.
The recommendation by JCIH in 2007 that AABR is the
most appropriate screening technology for infants who
received care in the NICU was made based on evidence of
the prevalence of neural hearing losses in that
population (Berg et al., 2005). There is concern that infants
with hearing thresholds less than 40 dB HL (common
screening intensity of AABR in the NICU) might be at
higher risk to be missed than those screened by OAE
technology (commonly used in well-baby nurseries). For
example, Johnson and colleagues (2005a)
demonstrated that as many as one in five newborns with
hearing thresholds between 25 and 40 dB HL using
behavioral testing at age nine months would have
potentially passed AABR screening as a newborn due to
the fact that their later-demonstrated hearing levels did
not rise to the 40 dB HL threshold of detection for this
screening technology. Similarly, Wood, Davis, and Sutton
(2013) found that one significant risk factor associated with
delayed-onset hearing loss could be defined as the infant
cared for in the NICU who did not pass OAE screening in
both ears, but subsequently passed rescreening in both
ears when performed with AABR technology. Finally, Berg
and colleagues (2005) recommended using AABR followed
by OAE screening to screen infants receiving care in the
9
NICU. Although the JCIH has not changed the existing
recommendation, screening with both technologies would
ensure that children with hearing thresholds in the mild/
moderate range would not be missed in the NICU.
Although use of both technologies in the NICU has
advantages and would detect both auditory neuropathy
and minimal-mild hearing thresholds with higher rates of
identification, there are a number of other factors to be
considered. First, there is an increased rate of transient
middle ear fluid among high-risk infants cared for in the
NICU (Hunter, Prieve, Kei, & Sanford, 2013). This finding
could result in a higher screen refer rate for diagnostic
testing. In addition, the change in JCIH recommendations
would require hospitals to purchase and monitor two
different pieces of equipment (although combination
devices have now come on the market), require additional
screener time, and impose higher costs on the hospital
and insurance. It could also mean additional stress for
families of infants with a transient conductive
component and a need for unnecessary diagnostic testing.
The objective of the current recommendation for continued
surveillance of both hearing skills and language
development in the medical home is to increase the
probability of identifying mild, progressive, and late onset
hearing loss (AAP Committee, 2017). However, some
infants will regrettably continue to be identified late (Holte
et al., 2012). States and hospitals will need to consider the
options and the costs in choosing the best technology and
protocols to implement.
Communication and Documentation of Results
Screening results should be conveyed immediately to the
family so that they can understand the outcome of the
screening and the importance of follow-up when indicated.
The use of the scripts developed by the state EHDI
program or the National Center for Hearing Assessment
and Management (NCHAM) is recommended (NCHAM,
n.d.). For the baby who does not pass the hospital screen,
the family should be informed in a manner that
maximizes the likelihood that follow-up will occur when
needed, a manner not overly stressful for the family, but at
the same time not suggesting overly-optimistic estimates
of the reasons for the failed outcome. To facilitate this
process for families, primary health care professionals
should work with EHDI team members to ensure that:
• Communications with families are confidential and
presented in a caring and sensitive manner, preferably
face-to-face.
• Educational materials are offered to families to provide
accurate information in an appropriate reading
level and in a language the families/caregivers are able
to comprehend with clearly-stated next steps
(Nicholson et al., 2016).
• Families are informed in a culturally-sensitive and
understandable manner that their infant needs further
hearing testing, and families are informed about the
importance of prompt follow-up (DesGeorges, 2017).
• Before discharge, an appointment should be made for
follow-up rescreening or for audiological testing.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
• Alternate contact information for a family should be
obtained prior to discharge to ensure timely follow-up
(Winston-Gerson & Hoffman, 2017).
Unfortunately, about one third of the time, infants who fail
their birth screen do not receive timely and appropriate
follow-up (Centers for Disease Control and Prevention
[CDC], n.d.-a; Holte et al., 2012). Accordingly, reducing
and preventing LTF/LTD starts with birth-hospital hearing
screening personnel and training. To facilitate the
screening process for primary care providers and better
ensure timely follow-up, EHDI systems should ensure that
medical professionals:
• Receive the results of the screening test (pass, fail, or
not completed) and the type of test administered (OAE
or AABR), as documented in the hospital medical
record. Although the type of screening test performed
has not typically been included in the report to the
primary care physician, including this information is now
recommended by the JCIH.
• Receive communication directly from the hospital for
each infant who does not pass the newborn
screening or leaves the hospital unscreened, with any
pertinent recommendations for follow-up included in the
communication.
Rescreening in the Outpatient Setting
A critical function in the success of UNHS is the ability to
ensure timely retesting for all infants who do not pass their
initial (birth) hearing screening. Outpatient rescreening
should be performed as soon as possible after hospital
discharge, and always before one month of age (or, in the
case of an older infant, as soon as possible following
discharge). The practice of multiple rescreenings in the
outpatient setting delays referral for diagnostic evaluation
and impacts timely diagnosis (White, Nelson, & Muñoz
2016). Rescreening should comprise a single valid
rescreen of both ears in the same session, regardless of
initial screening results, to ensure that fluctuation or
progression in hearing levels are not missed. Due to the
fact that hearing threshold levels between 25 and 40 dB
HL could be present at or near the threshold of the
technology being used, the outpatient rescreening should
always include the testing of both ears, even if only one
ear did not pass the inpatient screening. The outpatient
rescreen may be conducted by the birth hospital, by a
pediatric audiologist in the community, in the baby’s
medical home, or in an agency or clinic that adheres to
best-practice protocols with appropriate screening
equipment and demonstrable screener training and
competence. Regardless of who performs the
rescreening or the location in which the rescreening is
performed, there is an obligation to report outcomes of all
rescreening results whether pass or fail, to the state EHDI
program.
If the infant does not pass in one or both ears on the
rescreen, immediate referral to a pediatric audiologist
with capabilities for a diagnostic ABR testing should be
made. Preferably, the parent/guardian should be provided
10
with an appointment with the audiologist prior to leaving
the rescreening facility. When the outpatient rescreen is
performed by a pediatric audiologist, and the infant does
not pass the rescreen in one or both ears, it is preferable
that the diagnostic evaluation be initiated immediately (i.e.,
during the same appointment).
Outpatient hearing screening at no later than one month
of age should also be made available to infants who were
discharged before receiving the birth admission screening,
and to infants who were born outside a hospital or birthing
center in a location that does not provide newborn
hearing screening. Similarly, outpatient screening also
should be made available to the infant whose parents
previously declined or were unable to complete the
hearing screening and subsequently decided to have their
baby screened.
Rescreening in the Medical Home
Since the publication of the JCIH 2007 statement, an
increasing likelihood of OAE rescreening in the physician’s
office has been noted (Nelson, Bougatsos, & Nygren,
2008). Some primary care physicians have OAE screening
devices for use in the medical office, both for the purpose
of rescreening newborns (when indicated) and for
screening older children. The American Academy of
Pediatrics has published guidelines regarding rescreening
in the medical home (American Academy of Pediatrics,
2014a), and JCIH supports these guidelines. Specifically,
the guidelines for rescreening hearing, when performed in
the physician’s office, include the following highlights.
• Rescreening of infants must be performed using an
automated physiologic measurement (OAE or AABR),
not by assessing behavioral responses to
environmental sounds or noises (e.g., using whispered
speech or noisemakers).
• Physicians who rescreen in the medical office are
obligated to report rescreen outcomes (both pass and
fail results) to the state EHDI system.
• The equipment used for rescreening must be
calibrated, and annually re-calibrated, by the
manufacturer or other entity (e.g., special-instruments
distributor or hospital clinical engineering department).
• There must be a quiet environment for office-based
testing to avoid having children fail the rescreening
even if they have normal hearing.
• Office-based personnel who perform the rescreening
must be appropriately trained in the use of the
equipment.
• Infants who were hospitalized in the NICU and who did
not pass a hospital-based screening should be referred
directly to a pediatric audiologist and not rescreened in
the medical home, due to the increased likelihood of
hearing loss including auditory neuropathy (American
Academy of Pediatrics, 2014a).
• At the time of rescreening, both ears should be tested,
even if only one ear did not pass the screening
performed at the hospital.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Hospital based UNHS programs have proven efficacy due
to the ability to standardize processes and procedures
through the state EHDI programs, although, some
hospitals provide rescreening and some do not. A
shortcoming of most hospital record-keeping entries is that
the technology used at the time of hospital-based screen-
ing is often not recorded in the hospital discharge
summary, and the primary care provider may have some
difficulty obtaining such information. However, some state
database systems are now designed or are being
expanded to require identification of the type of equipment
used for the birth screen. JCIH does not support providers
performing the initial newborn hearing screening in the
office, but rather supports the positions summarized that
follow, as outlined by a number of AAP publications (AAP,
2014a, 2014b; AAP Committee, 2017). Primary healthcare
providers should become very familiar with these
guidelines.
The AAP does not support the concept of performing the
initial newborn hearing screening test in the medical home
rather than at the hospital (American Board of Audiology
[ABA], 2016). The responsibility of the medical home is to
refer infants for further testing if needed. Newborn hearing
screening has been successfully implemented over the
past two decades, in part because over 95 percent of
newborns are delivered in a hospital and have immediate
access to a hospital-based program to perform a
physiologic test to screen for hearing. The success of
these programs is due in part to the captive audience of
newborns and has resulted in an efficient, cost-effective
implementation with greater standardization of protocols,
technology, and accuracy. In addition, the hospital-based
institutional commitment to equipment calibration and
oversight by qualified audiologists in the hospital setting
allows for a quality standard that may be difficult to
duplicate when screening is performed in the medical
office setting.
The AAP recommends the first newborn hearing screening
test be completed at the birthing hospital (AAP, 2014a);
however, there may be an occasional situation when this
is not possible. Examples include infants born at home
and not screened by the midwife or birth attendant, infants
whose parents decline hospital-based screening but later
realize the merits of screening and consent to office-based
screening, and infants who were inadvertently missed at
the hospital for any reason. If, on these rare occasions, the
first newborn hearing screen is performed in the medical
office, all of the guidelines concerning equipment needs,
screening techniques, follow-up, and reporting of results to
state entities would apply (AAP, 2014a, 2014b).
Improving EHDI Loss-to-Follow-Up/Loss-to-
Documentation Rates
Hearing screeners in the hospital or medical home, and
state EHDI coordinators should be aware of some of the
following situations under which infants may be lost to
the UNHS system (American-Speech-Language-Hearing
Association [ASHA], 2008b):
11
 • Home births and other out-of-hospital births:
◊ States should develop a mechanism to
systematically offer newborn hearing screening for all
out-of-hospital births.
• Across-state-border births:
◊ States should develop written collaborative
agreements among neighboring states for sharing
hearing-screening results and follow-up information.
• Hospital screenings that are not completed prior to
discharge:
◊ When infants are discharged before the hearing
screening is performed, a mechanism should be in
place for the hospital to contact the family and
arrange for an outpatient hearing screening.
• Transfers to in-state or out-of-state hospitals:
◊ Discharge and transfer forms should contain the
information regarding whether a hearing screening
was performed and the results of any screening.
◊ The recipient hospital should complete a hearing
screening if one was not previously performed, or if
there is development of a new risk factor for being
deaf or hard of hearing.
A pathway for initial screening for infants who, for a variety
of reasons, miss the initial screening in the birth hospital
should be developed and followed such that no infant will
be inadvertently missed, either in the birth hospital, subse-
quent hospital(s), or after a home-birth. See the National
Institute for Children’s Health Quality website (NICHQ,
2016) for more information and suggested pathways.
Pediatric Diagnostic Audiology
Audiologic diagnosis of the infant is the sole purview of
the audiologist with specific skills, knowledge, and access
to all necessary equipment for infant and early childhood
audiologic diagnostic evaluations. It is incumbent upon the
audiologist who lacks experience or equipment to refer
infants to audiology centers where timely and
comprehensive evaluation can be accomplished. Only
through consultation with such an audiologist can accurate
diagnosis occur, and timely early intervention for the infant
and family be assured. Pediatric audiologists and facilities
can be discovered through the roster of Pediatric Board
Certified audiologists on the ABA website
(http://www.boardofaudiology.org) and the Early Hearing
Detection and Intervention–Pediatric Audiology Links to
Service (EHDI PALS) website (http://www.ehdi-pals.org).
For more information about the searchable EHDI PALS
facility directory, see Chung and colleagues (2017).
Key Components of an Audiologic Diagnostic
Evaluation in an Infant
Audiologic diagnosis in the infant must be conducted
in a timely manner by audiologists skilled in infant
assessment with access to all necessary equipment using
evidence-based protocols. Testing is performed to quantify
frequency-specific thresholds for air- and bone-conduction
stimuli, and to determine the type and degree of hearing
loss in each ear to guide the fitting of hearing aids.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
In keeping with the 1-3-6 (or 1-2-3) EHDI goals, audiologic
diagnosis should be completed no later than 2–3 months
of age. This earlier age facilitates the diagnostic process
as infants are more likely to sleep for prolonged periods
of time required to complete all measures. In children with
special health needs, delay in diagnosis of hearing loss
may be unavoidable due to attention paid to other health/
time-urgent diagnostic and treatment procedures; however,
every effort should be made to minimize the delays. When
possible, audiologists can evaluate infants in the NICU,
pediatric intensive care unit, or in conjunction with
examinations or procedures conducted with general
anesthesia or sedation.
The key aspects of audiologic assessment for infants and
young children are:
• Auditory brainstem response is the gold standard
test for threshold estimation for infants and children
who cannot complete behavioral audiologic
assessment. ABR provides ear- and frequency-specific
threshold estimates that are necessary for the
diagnosis of the type, degree, and configuration of
hearing loss and provision of amplification (Gorga et al.,
2006).
• Measures of middle ear function should be completed
as part of the diagnostic audiologic process for infants
and young children. Either tympanometry or wideband
reflectance can be used to characterize middle ear
function (Hunter et al., 2013).
• Acoustic reflexes are an important test of middle ear
function and the integrity of auditory brainstem path
ways (de Lyra-Silva et al., 2015).
• Otoacoustic emissions provide important information
about the integrity of the outer hair cells of the
cochlea and provide critical information about the
differential diagnosis of auditory neuropathy spectrum
disorder and sensorineural hearing loss (Gorga et al.,
2000).
• Behavioral assessment of hearing is the gold standard
for estimation of hearing thresholds. Visual
reinforcement audiometry (VRA; for infants 6–24
months; Widen et al., 2005) and condition play
audiometry (CPA; for toddlers 24+ months; Norrix,
2015) are established methods based on conditioned
responses to sound.
The following section provides additional detail about the
research regarding diagnostic audiological assessment for
infants and young children.
Auditory brainstem response evoked potentials using
click and frequency-specific stimuli. Auditory brainstem
response for estimation of hearing thresholds is the
gold-standard for determination of hearing thresholds
under 6 months of age. Because ABR is not a test of
hearing but rather a measure of an electrophysiologic
response to auditory stimulation, confirmation of hearing
(perception) requires behavioral evaluation as soon as
the child is developmentally capable of providing reliable
and valid behavioral responses to sound. It is theoretically
12
possible that an infant can have normal ABR recordings,
yet not be able to perceive or understand the signal, since
comprehension occurs at a higher level in the brain than
the sites from which an ABR is recorded.
In the diagnostic ABR, recording of electrophysiologic
response requires that the newborn or infant sleep
soundly for a prolonged period of time so that quiet
responses, unmarred by artifact and noise, can be
obtained. In some cases, sedation or anesthesia is
required to ensure sufficient quiet time for all diagnostic
measures to be completed; however, in the young infant
with appropriate preparation, natural sleep recordings are
quite feasible. Frequency-specific (toneburst) stimuli are
used to elicit neural responses that enable determination
of thresholds, and form the foundation for determining
hearing aid amplification characteristics. Thresholds for
both air-conducted and bone-conducted stimuli are
measured to determine type (i.e., conductive,
sensorineural, mixed) of hearing loss. Bone conduction
thresholds are necessary to estimate additional hearing
aid gain and output if there is a conductive component.
When the ABR shows no response, a specialized protocol
(high-intensity click stimulus at positive and negative
polarities) should be completed to assess possible auditory
neuropathy. The hallmark of the auditory neuropathy ABR
is a prominent cochlear microphonic that follows the
stimulus polarity when it is reversed. Waveforms
subsequent to the polarity-reversing cochlear microphonic
are typically absent or significantly aberrant (e.g., poor-
ly-defined, delayed and/or low-amplitude subsequent
waveforms). This ABR protocol is the only-recognized and
substantiated method for determining auditory neuropathy
(Starr, Picton, Sininger, Hood, & Berlin, 1996).
Research has indicated a good correlation between ABR
toneburst thresholds and behavioral thresholds in the
middle to high frequencies in infants and young children
(Gorga et al., 2006; McCreery, Kaminski, et al., 2015). For
infants and young children displaying sensorineural
hearing loss, toneburst ABR hearing threshold estimates
range from 5 dB better to 5 dB poorer than the nominal
intensity, depending on the stimulus presentation
intensity and frequency. A single correction factor for
predicting hearing threshold from ABR threshold estimates
is less accurate than use of a level-dependent correction
factor (McCreery, Kaminski, et al., 2015). For example,
ABR results at lower intensities tend to over-estimate
hearing thresholds (suggest hearing loss when there is
none), while ABR threshold estimates at higher intensities
tend to under-estimate the hearing thresholds (suggest
hearing is better than it actually is). In keeping with the
cross-check principle, while toneburst ABR serves as the
basis for the initial hearing aid fitting, it should be
accompanied by ear-specific and frequency-specific
behavioral response hearing testing using a VRA protocol
(conditioned response) beginning at approximately four to
five months of age, depending on the infant’s
developmental status (Widen et al., 2005).
When VRA is conducted according to careful stimulus,
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
response, and conditioning paradigms, valid and reliable
thresholds can be obtained from the typically developing
infant. Despite the correlation in average ABR threshold
responses to behavioral hearing thresholds across all
infants, individual differences vary, and for this reason,
validation of ABR thresholds by behavioral testing should
occur at the earliest opportunity. Further, since it is not
uncommon that children exhibit progressive hearing loss
in the first months and years of life, on-going audiological
evaluation is essential for any child who is at risk for
hearing loss, or any child who wears hearing aids.
Although toneburst ABR is the gold-standard for estimating
hearing thresholds in the infant, other evoked-response
protocols, stimuli, and technologies are emerging that
demonstrate frequency specificity, as well as equivalent,
if not superior test efficiency (e.g., ABR or Automated
Steady-State Response [ASSR] using puretone or
broadband [chirp or click-evoked-chirp stimuli], Cebulla &
Elberling, 2015; Cebulla, Lurz, & Shehata-Dieler, 2014).
Any technology, protocol or stimulus used for objective
determination of frequency-specific hearing thresholds
should be rigorously and independently validated for the
ability to accurately predict behavioral hearing thresholds
in infants and young children of all ages and all types and
degrees of hearing loss.
Use of novel stimuli (brief-tone chirp or click evoked
[CE]-chirp) has recently received attention as a potential
alternative to click and toneburst stimuli, with reported
improvements in frequency-specificity and shortened test
duration. Recordings can be made of multiple frequencies
simultaneously, and stimuli can be presented binaurally.
Binaural presentation, however, decreases the amplitude
of the response, and may not be indicated for use in a
child with bilateral hearing loss (Cebulla et al., 2014; Ferm,
Lightfoot, & Stevens, 2013). In a study comparing ABR
response amplitudes for tone-pip stimuli at four
frequencies (500, 1000, 2000, 4000 Hz) to narrowband
CE-Chirps at corresponding frequencies, authors reported
increased amplitude with fewer stimulus presentations in
infants with normal hearing (Cebulla et al., 2014; Ferm et
al., 2013; Stuart & Cobb, 2014). Use of a correction factor
was proposed to increase the accuracy of estimating
hearing thresholds.
Van Maanen and Stapells (2010) observed that there are
few studies of deaf and hard of hearing infants and young
children comparing ASSR thresholds to ABR thresholds.
Results of their study demonstrated that hearing
thresholds in children could be reliably classified as normal
or elevated based on ASSR thresholds. It was noted,
however, that there are insufficient data comparing deaf
and hard of hearing infants with typically hearing infants
and young children using both air- and bone-conduction
stimuli. Higher thresholds are seen in preterm infants as
compared with full-term infants. This gap resolved by age
18 months, suggesting auditory maturation of preterm
infants (Sousa, Didoné, & Sleifer, 2016). The ability of
ASSR to distinguish between normal hearing and mild
13
hearing thresholds is limited (Sousa et al., 2016). There is
greater variability in ASSR threshold estimates in infants
with typical hearing, such that a standard correction factor
cannot be generated (Alaerts, Luts, Van Dun, Desloovere,
& Wouters, 2010). Additionally, the mean air-bone gap for
low-frequency ASSR thresholds is significantly greater
than that for behavioral (visual reinforcement audiometry),
with wide variations across infants (Casey & Small, 2014).
Accurate estimates of bone-conduction thresholds as
elicited by ASSR have not been reported (Casey & Small,
2014). As studies are published that examine these
relationships over a variety of ages, hearing thresholds,
and etiologies, future endorsement may be possible.
Limited data are currently available regarding the
relationship between behavioral hearing thresholds and
chirp-elicited responses in infants with a variety of types
and degrees of hearing loss, warranting further study.
Given the substantial literature that supports the sensitivity
and specificity of toneburst ABR in estimating type and
degree of hearing loss in infants, alternative test protocols
must demonstrate distinct advantages to be considered
viable alternatives. Such advantages would need to
include improved accuracy in auditory threshold estimation
across all ages, types and degrees of hearing loss, and
reduced duration of testing time.
Emerging data suggest that new approaches to ABR
recording, such as using specialized filtering, advanced
signal processing techniques, and placing the pre-amplifier
at the position of the electrode, may improve the
signal-to-noise ratio in children who are not soundly
sleeping. Limited data exist demonstrating the validity of
frequency-specific hearing levels obtained through the
use of these techniques in non-sedated/awake recordings
(Cone & Norrix, 2015). However, independent evidence is
insufficient at this time for the JCIH to endorse this
methodology for acquisition of reliable and valid ABR or
ASSR threshold estimates in a child of any age who is
moving, vocalizing, or otherwise not relatively quiet and
still.
Middle ear measures. Tympanometry, wideband
reflectance. In the diagnostic audiologic evaluation,
measures of middle ear movement assist in the
differentiation of conductive and sensory or neural sites.
At the time of newborn hearing screening, neonates may
have retained amniotic fluid in the middle ear space,
resulting in a not-pass outcome. The standard measure for
detecting middle ear fluid has long been high frequency
tympanometry, due to superior sensitivity and specificity
in detecting middle ear fluid or effusion in infants as
compared with standard 226 Hz tympanometry. Use of
the 1000 probe tone is recommended up to age 9 months
(Hoffmann et al., 2013).
Increasingly, wideband reflectance, rather than
tympanometry is being studied and used with neonates,
due to reported superior sensitivity and specificity. Prieve,
Vander Werff, Preston, & Georgantas (2013) noted that
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
conductive hearing loss can be detected in infants using
either 1000 Hz tympanometry or wideband reflectance.
Reflectance measures are sensitive to transient middle-ear
conditions in infants who did not pass birth screening and
subsequently passed screening at age 1 month (Voss,
Herrmann, Horton, Amadei, & Kujawa, 2016).
Wideband reflectance is sensitive to middle-ear disorders
including otitis media with effusion in infants and children,
and has shown high test–retest reliability demonstrated
by high interclass correlations (Hunter, Tubaugh, Jackson,
& Propes, 2008). Keefe and Simmons (2003) analyzed
wideband reflectance obtained from a two-stage newborn
hearing-screening protocol (OAE/ABR), which resulted in
a 5% false-positive rate. Wideband reflectance measures
demonstrated that 80% of the OAE screening referrals had
abnormal responses, indicating evidence of
middle-ear dysfunction. Another study of OAE screening
in infants showed significantly higher reflectance between
0.63 and 2 kHz in those infants who failed, compared with
those who passed OAE screening (Vander Werff, Prieve,
& Georgantas, 2007). The increasing use of wideband
reflectance rather than tympanometry to detect middle ear
effusion, suggests that this technique may supplement, if
not supplant, tympanometry in the coming years (Hunter et
al., 2008).
Acoustic reflex thresholds. Measurement of acoustic
reflex thresholds is completed using a 1000 Hz probe-tone
for newborns and infants under 9 months of age (de Lyra-
Silva, Sanches, Neve-Lobo, Ibidi, & Carvallo, 2015). The
acoustic reflex can be reliably measured in infants with
normal tympanograms, and can assist in the diagnosis of
peripheral and neural hearing involvement (de Lyra-Silva
et al., 2015). Good reliability has been shown for tonal and
broadband stimuli (Kei, 2012), with published normative
data. It is important to recognize that in the infant, the
intensity of the stimulus tone or noise will be greater than
the dial setting, as the infant ear canal is considerably
smaller than the standard coupler used for calibration.
Normative data for acoustic stapedial reflexes in healthy
neonates demonstrated that mean reflexes occurred at 57
dB HL for broadband noise, and ranged from 65-81 dB HL
for tonal stimuli (Kei, 2012). As such, caution must be used
in setting the upper limit of stimulus intensity used in
eliciting the reflex. The acoustic reflex test is particularly
helpful in cases where auditory neuropathy is
suspected, as the reflexes are expected to be absent.
Berlin and colleagues (2005) found that absent or elevated
middle ear muscle reflexes in the presence of normal
otoacoustic emissions and confirms auditory neuropathy.
Otoacoustic emissions. OAE (distortion product or
transient evoked) testing is essential in the pediatric
diagnostic evaluation (Holte et al., 2012; Norton et al.,
2000a; Prieve, Schooling, Venediktov, & Franceschini,
2015). OAEs are measureable sounds that occur when the
cochlea is stimulated with a low-intensity click or puretone
stimuli. The OAEs are recorded via a probe assembly with
a microphone, placed in the external ear canal. Diagnostic
14
OAEs provide information about the presence/absence of
outer hair cell function, from which hearing level (typical
vs. elevated) can be inferred, when the middle ear has
been shown to be free of effusion. Although it is possible to
have OAEs in the presence of mild sensory hearing loss,
the magnitude of the emission diminishes with increasingly
elevated thresholds, and the emissions are not observed
in hearing thresholds greater than 30–35 dB HL. Mild
degrees of hearing loss are difficult to define using OAE
technology; however, DPOAEs accurately separate normal
hearing ears from those with moderate and greater
degrees of hearing loss (Gorga et al., 2000). The
magnitude (intensity or amplitude) of an infant OAE is
greater than in adults, and as such, the detection of the
emission is facilitated. Generally, infants with present
DPOAEs are predicted to have hearing thresholds better
than 30 dB HL. Infants with absent DPOAEs (in the
presence of normal tympanometry or wide band
reflectance) are predicted to have hearing thresholds
poorer than 30 dB HL (American Academy of Audiology
[AAA], 2011, 2012). The OAE assessment is not sufficient
for determining hearing thresholds, and cannot be used
in isolation to determine hearing aid specifications. It is
important to remember that the otoacoustic emission only
reflects activity in the cochlea. Infants with auditory
neuropathy or more central auditory pathologies are
expected to have a normal OAE, yet clearly do not have
normal auditory function.
Assessing infants and young children who cannot
be evaluated using behavioral testing. Approximately
40% of young children who are deaf or hard of hearing
have coexisting conditions that, in some cases, may
render audiologic evaluation challenging (e.g., physical,
intellectual, psychological, or emotional needs or barriers;
Gallaudet Research Institute, 2013). In the hands of
the pediatric audiologist, many of these children can be
conditioned to provide valuable behavioral indications
of hearing levels. However, some children may never
become candidates for conventional/behavioral
testing methods for the purpose of determining and
validating frequency-specific hearing thresholds. After
determination by a pediatric audiologist that behavioral
testing cannot yield meaningful audiometric information,
electrophysiological testing with sedation or anesthesia
may be indicated, if there are no medical contraindications
and if the results of the evaluation will influence the
treatment or management of the child. Periodic
monitoring of hearing status to assess possible delayed-
onset or progressive hearing loss (e.g., congenital
Cytomegalovirus [CMV]) may also require continued use
of electrophysiological measurements. This creates a
potential dilemma when anesthesia is required for initial
and/or repeated ABR measurement for the purpose of
diagnosis and monitoring. Recent research has shown
the potential risk to cognitive function in the young child
who undergoes general anesthesia (Sun et al., 2016).
The benefits and risks must be carefully weighed and the
JCIH recognizes that medically fragile children may not be
candidates for anesthesia.
If the child is unable to be evaluated using conventional
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
behavioral tests and ABR evaluation using anesthesia is
required, it is important to determine whether the outcome
of the ABR evaluation will impact treatment decisions (e.g.,
hearing aid fitting, cochlear implantation, communication
approaches). If the results of the ABR evaluation under
anesthesia are not expected to change the course of
treatment, it is reasonable to question the necessity for
undergoing a costly and time-consuming procedure that
carries with it a measure of risk. Professional judgment as
well as input and observations from the family are
necessary to determine if use of amplification and/or other
assistive technology has a probable chance of enhancing
access to auditory information. Communication is
inherent in the decision to provide amplification and to
monitor its function, use, and benefit. Successfully
maximizing auditory access to sound requires diligence
in communication with the family and other professionals
involved in the child’s intervention plan. Close monitoring
provides information about whether hearing sensitivity is
stable, fluctuating, or decreasing.
Assessing infants/toddlers with middle ear effusion or
retained amniotic fluid. It is not uncommon for middle ear
effusion or retained amniotic fluid in the middle ear to
persist in infants regardless of hearing status, causing
referral from newborn hearing screening and/or a
(temporary) conductive hearing loss. Management of
middle-ear fluid in the infant should be coordinated by the
infant’s pediatrician/primary-care provider and/or a
pediatric otologist, with the audiologist’s input, and in
conjunction with the family’s preferences. In some cases,
myringotomy with or without a pressure equalization tube
placement will be necessary to complete the diagnostic
evaluation in a timely fashion during the critical
developmental period of early infancy (Rosenfeld et al.,
2013). Middle ear effusion often complicates and delays
the diagnostic process, resulting in delayed diagnosis of
hearing loss.
There is increasing evidence that the infant with retained
amniotic fluid and/or persistent middle ear fluid is at
increased risk for on-going middle ear involvement (Doyle,
Kong, Strobel, Dallaire, & Ray, 2004). It is important,
however, to recognize that not all conductive hearing loss
is attributable to middle ear fluid. Congenital ossicular
abnormalities can be present and can be distinguished by
the audiologist using a combination of air- vs. bone-
conduction threshold estimation, in addition to
tympanometry, acoustic reflex threshold tests, and OAE
testing; as well as the otologist’s examination and
radiographic studies (Boone, Bower & Martin, 2005).
In 2013, the JCIH recommended that children at-risk for
delayed speech and language development due to chronic
middle ear conditions receive intervention services. When
present from the time of birth, if a conductive hearing
loss (or the conductive component of mixed hearing loss)
cannot be medically remediated by six months of age, the
child should be considered for hearing aid amplification,
communication supports, and referral to early intervention
services, even if these services may be short-term. Such
15
interventions are necessary to address and prevent
developmental language delays. This type of situation
commonly occurs when providing care for infants with cleft
palate or Down Syndrome.
Assessing the infant/toddler with auditory neuropathy.
Auditory neuropathy may occur in association with specific
conditions (i.e., extreme prematurity, hyperbilirubinemia,
hydrocephalus), or it may present in infants where no
obvious causative factor is identified (Kim, Choi, Han, &
Choi, 2016). Audiological characteristics include the
presence of OAEs and/or cochlear microphonics
accompanied by poor morphology of the ABR and absent
or elevated middle ear muscle reflexes (Hood, 2015).
Puretone audiometry, when the infant is developmentally
able to participate in behavioral testing, is typically
characterized by bilateral or unilateral fluctuating or stable
thresholds which may range from mild to profound
(Attias & Raveh, 2007; AAA, 2013). Some of these children
may have severe neurological involvement and may not
ever be able to participate in behavioral evaluation (Uhler,
Heringer, Thompson, & Yoshinaga-Itano, 2012).
Until such time that reliable behavioral thresholds can
be determined and hearing status appears to be stable,
infants with auditory neuropathy may require frequent
audiological monitoring to assess progression,
improvement, or fluctuation in electrophysiological
threshold estimates. ABR monitoring, however, cannot be
used to determine or to demonstrate change in hearing
sensitivity, as the hallmark Wave V is not present in ABR
recordings of infants or children with auditory neuropathy.
Reversal or resolution of auditory neuropathy can occur for
diverse reasons including resolving hyperbilirubinemia or
decreased intra-cranial pressure (Attias & Raveh, 2007).
Hearing aid amplification should be deferred in children
with auditory neuropathy until minimum responses or
behavioral hearing thresholds can be established (AAA,
2013). Although Wave V of the ABR is known to correlate
with hearing thresholds in cases of conductive or
sensorineural hearing loss, the absence of this evoked
response in infants with auditory neuropathy poses a
challenge in programming hearing aid gain and output
relative to a validated hearing aid prescriptive protocol
(e.g., Desired Sensation Level; Seewald, Moodie, Scollie,
& Bagatto, 2005). This practice can result in significant
over-or under-fitting of hearing aid amplification for infants
with auditory neuropathy. When the child is functioning at a
developmental age in which reliable behavioral thresholds
can be determined, appropriate hearing aid settings can
be established and hearing aids fitted.
Cortical Auditory Evoked Potentials (CAEP) are
increasingly being investigated to measure detection and
discrimination of sound (Cardon & Sharma, 2013; Cone
& Whitaker, 2013; Sharma, Cardon, Henion, & Roland,
2011). For the infant with auditory neuropathy where there
is no ABR response to determine auditory threshold, the
CAEP can be measured to demonstrate auditory
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
awareness at the cortical level. Auditory awareness and
discrimination of tonal and speech stimuli can be
measured by scalp-recordings of primary auditory
cortical responses. CAEPs are now beginning to be used
as biomarkers of acoustic threshold and speech perception
capacity in children with auditory neuropathy (Campbell,
Cardon, & Sharma, 2011). Recording of CAEPs can yield
frequency-specific threshold information for about 50% of
children with auditory neuropathy (Cone & Whitaker, 2013;
He et al., 2013). Although ABR thresholds closely correlate
with hearing thresholds, there can be variability in the
accuracy of the toneburst ABR threshold estimate in
predicting true behavioral hearing thresholds of individual
children at specific frequencies (particularly low
frequencies). For some children, toneburst ABR
threshold estimates will be almost identical to later
behavioral thresholds while other children may have
differences that would result in over- or under-
amplification at specific frequencies. For this reason,
validation of ABR thresholds by behavioral testing should
occur at the earliest opportunity.
Assessing the infant/toddler using behavioral
audiometry. Although physiologic and electrophysiologic
measures are the most reliable and valid estimates of
hearing in the neonate and young infant, it is only through
evaluating behavioral responses to sound that a true
measure of hearing is made. It should be noted that Be-
havioral Observation (sometimes termed BOA or
Behavioral Observation Audiometry) is not a conditioned
response, and as such, does not yield reliable and valid
hearing thresholds on which to base hearing aid selection
characteristics. BOA plays a role in the cross-check
principle, in that a child who reliably startles to loud sound
is unlikely to have profound bilateral hearing loss. A child
who does not startle to loud sound, however, cannot be
assumed to have a peripheral hearing loss. Because BOA
is not a conditioned response, but rather an observation of
behavior that follows exposure to sound, it cannot be used
to make diagnostic statements about hearing sensitivity
(AAA, 2012).
Puretone thresholds are recognized as the gold standard
for determining hearing status (Prieve et al., 2013). VRA
is a conditioned response and uses the development of
auditory localization in the horizontal plane to observe
and reinforce head-turn behavior in response to puretone
and speech stimuli. When VRA is conducted according
to careful stimulus, response, and conditioning protocols,
valid and reliable results can be obtained from the typically
developing infant (Widen & Keener, 2003). Audiometric
threshold estimates obtained via VRA are referred to as
Minimum Response Levels as they reflect the lowest
intensity level at which a response is observed (Widen &
Keener, 2003).
For the slightly older child (24+ months, assuming typical
development), conditioned play audiometry provides
reliable and valid thresholds to speech stimuli, as well as
air- and bone-conducted puretone stimuli. Further, word
16
and speech perception are tested to provide information
about how the child recognizes and discriminates speech
stimuli. It is only through behavioral measures that a true
picture of hearing can be obtained. Although physiologic
and electrophysiologic measures that underlie hearing
provide invaluable information in the child who is too
young to be tested through behavioral means, perception
and understanding of acoustic stimuli can only be
measured using behavioral tools (Widen & Keener, 2003).
Infant audiologic assessment and determination of hearing
thresholds require not only adherence to best-practices,
but also sufficient time, space, skill, appropriate equipment
and protocols, and patience.
Referrals
If the referral for the pediatric diagnostic audiology
evaluation did not originate with the infant’s primary care
provider (PCP), a copy of the diagnostic audiology report
should be sent to the PCP with recommendations for
medical and otologic evaluations (AAP Committee, 2017;
AAP, 2014a, 2014b) and the state EHDI program CDC,
2016a). In addition, a referral to the state Part C early
intervention program must be made upon confirmation of
a child being deaf or hard of hearing. Although the Part C
revised guidelines state the referral must be made within
seven days, immediate referral with a goal of 48 hours is
recommended by JCIH in the early intervention best
practices document (JCIH, 2013). Based on the 1-3-6
guidelines, referral to Part C should always be completed
as soon as a child is diagnosed as deaf or hard of hearing,
and always prior to six months of age. Diagnosis does not
imply that thresholds are determined for all test
frequencies, but rather, based on key frequencies (e.g.,
500 Hz and 2000 Hz), it can be shown through air- and
bone-conduction testing that probable permanent
threshold elevation exists in one or both ears.
Medical Evaluation
Completion of a comprehensive medical evaluation is
imperative, concurrent with or shortly following
confirmation and/or diagnosis of hearing thresholds (mild,
moderate, severe, or profound levels) outside the typical
range. The purpose of the medical evaluation for children
confirmed as deaf or hard of hearing is to attempt to
determine the etiology, to identify related conditions, to
provide medical/surgical recommendations and
treatments, to provide referrals for indicated ancillary
services, and to collaborate with the family in informed
decision making for their child (Prosser, Cohen, &
Greinwald, 2015). Essential components of the
comprehensive medical evaluation are a detailed history
comprising hearing screening details including type (OAE
or AABR), timing (age of infant at time of screen or
diagnostic test), and number of screens completed; family
perspectives on auditory awareness and responses of
their infant; pregnancy and obstetrical history; and a family
history, especially as it relates to family members who
have been deaf or hard of hearing. Some targeted
questions to identify a variety of syndromes associated
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
with children who are deaf or hard of hearing can also be
useful. Copies of the diagnostic pediatric audiology test
results, both objective and behavioral, should be
requested, including whether these objective measures
were obtained with or without sedation.
A physical examination should also be performed, and
radiologic and laboratory studies should be considered,
consistent with best practices as shown in the 2010
American Academy of Pediatrics Early Hearing Detection
and Intervention Guidelines for Medical Home Providers
(see https://tinyurl.com/y5zzowco). In addition, every
infant confirmed as deaf or hard of hearing, with or
without middle ear dysfunction, should be referred by the
medical home for specialty evaluations including otologic
evaluation, genetics evaluation, ophthalmologic evaluation,
and additional medical evaluations as indicated (AAP,
2010; Prosser et al., 2015). However, these referrals
should not delay the fitting of amplification, which should
occur as soon as sufficient audiological information is
obtained. It is important that medical professionals be
sensitive to the attitude, mood, and perspective of the
family when caring for the child who is newly identified
or diagnosed as deaf or hard of hearing. The health
professional should be prepared to refer the family for
counseling and participation in family support groups, in
addition to necessary medical, audiological, and early-
intervention services, with the goal that the infant and the
infant’s family receive optimal family-centered care (Arnold
et al., 2006; Davis et al., 2006).
Role of the Pediatrician/Primary Care Provider
The pediatrician or other primary care provider is
responsible for monitoring the general health,
development, and well-being of the infant. The PCP plays
an important role in monitoring birth hearing screening
outcomes, and ensuring follow-up with rescreening and
audiologic diagnostic evaluation when indicated. The PCP
is part of the team that ensures that the audiologic and
medical assessment is conducted in a timely fashion for
infants who do not pass screening. Rescreening
guidelines are available on the American Academy of
Pediatrics EHDI website in addition to other resources
(AAP, 2010; AAP, 2014a, 2014b; AAP Committee, 2017).
For all infants suspected or confirmed to be deaf or hard of
hearing, the PCP must partner with other specialists,
particularly the otolaryngologist, audiologist, geneticist/
genetics counselor, and early intervention specialist to
facilitate coordinated and comprehensive care for the
infant and family. In 2010, AAP developed the
medical-home algorithm (guidelines) for management of
infants suspected or confirmed as deaf or hard of
hearing (see https://tinyurl.com/y5zzowco).
Middle-ear status should be monitored by the PCP,
because the presence of middle-ear effusion has the
potential to delay diagnosis of hearing and can further
compromise hearing. Surveillance of both middle ear
status and developmental milestones is recommended,
regardless of the infant’s birth hearing screening results or
17
hearing threshold levels (Rosenfeld et al., 2013). Prompt
specialty referrals should follow when new or
delayed-onset conditions are suspected (Gracey, 2003).
Because approximately forty percent of children confirmed
as deaf or hard of hearing will demonstrate additional
conditions or delays such as autism, blindness, learning
differences, genetic syndromes, et cetera, health care
providers have an important role in confirming that these
children are receiving comprehensive services (Roizen et
al., 2014). Regardless of the newborn hearing screening
results, the pediatrician or PCP should review every
infant’smedical and family history for the presence of
known risk indicators that require monitoring for delayed-
onset or progressive hearing loss (see Table 1). The PCP
should also ensure that an audiologic evaluation is
completed for these children as recommended (Coenraad,
Goedegebure, van Goudoever, & Hoeve, 2010; Fligor,
Neault, Mullen, Feldman, & Jones, 2005; Fowler, 2013;
Nance, Lim, & Dodson, 2006). In addition, the PCP is
responsible for ongoing surveillance of family concerns
about speech, language, hearing, auditory skills, and
developmental milestones of all infants and children
regardless of risk status, as outlined in the pediatric
periodicity schedule published by the American Academy
of Pediatrics (AAP Committee, 2017).
A growing body of research indicates that children who
receive cochlear implants (CI) are at increased risk for
developing bacterial meningitis over the general
population (Biernath et al., 2006; Gluth, Singh, & Atlas,
2011; Melton & Backous, 2011; Parner et al., 2007).
Historically, cochlear implant devices that specifically
involved a separate electrode positioner appeared to
confer a much higher risk of meningitis over other devices,
but have since been eliminated from the market. Starting
in 2002, the CDC established guidelines for additional
immunizationagainst bacterial meningitis in children with
cochlear implants, to be implemented in addition to
already-established routine prophylactic vaccinations
recommended for all children. The current
recommendations of the CDC and FDA vaccine programs
are available on the CDC website (CDC, 2016b).
Role of the Otolaryngologist
Otolaryngologists are physicians and surgeons who
diagnose, treat, and manage a wide range of diseases of
the head and neck, including ear, hearing, and vestibular
disorders. They perform a full medical diagnostic
evaluation of the head and neck, ears, and related
structures, including a comprehensive history and physical
examination, leading to a medical diagnosis and
appropriate medical and surgical management
(Rutherford, Lerer, Schoem, & Valdez, 2011). The
otolaryngologist’s evaluation includes a comprehensive
history to identify the presence of risk factors for
congenital or delayed-onset childhood hearing loss (see
Table 1; Morzaria, Westerberg, & Kozak, 2005), and a
physical examination that includes examination of the head
and neck structures for defects of the auricles (microtia);
patency of the external ear canals (atresia); status of the
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
tympanic membranes and middle ear structures; presence
of preauricular pits or cysts; positioning of eyes and orbits,
cheek bones, and jaws; and evidence of facial asymmetry.
Additional findings associated with congenital deafness
and elevated hearing thresholds include focal hair
discoloration with wide-appearing eyes (dystopia
canthorum), or different colored eyes. An evaluation for an
enlarged thyroid gland should also be performed.
Relatively common syndromes associated with
observable physical anomalies and permanent
sensorineural hearing loss include: Waardenburg
syndrome, CHARGE syndrome, VACTERL syndrome,
branchio-oto-renal syndrome, and Pendred syndrome
(Martini, Calzolari, & Sensi, 2009). Usher syndrome is
associated with progressive blindness secondary to
retinitis pigmentosa, a degenerative eye disease, and is a
common cause of deaf-blindness (Kimberling et al., 2010).
Congenital permanent hearing loss or transient/fluctuating
conductive hearing thresholds may be associated with
craniofacial anomalies such as cleft lip and/or cleft palate
(Nicholson, Christensen, Dornhoffer, Martin, & Smith-
Olinde, 2011). Children with craniofacial abnormalities,
despite the absence of middle ear fluid, may have
permanent conductive hearing threshold changes
secondary to abnormalities of the ossicular chain or
Eustachian tube anatomy/physiology. Tympanic membrane
visualization may reveal middle ear abnormalities such
as a congenital ossicular deformity, middle ear effusion,
or cholesteatoma. Syndromes associated with conductive
hearing loss might include: Down syndrome, Treacher-
Collins syndrome, Crouzon syndrome, Klippel-Feil
syndrome, branchio-oto-renal syndrome, CHARGE
syndrome, VACTERL syndrome, and Goldenhar syndrome
(Kimberling et al., 2010; Nicholson et al., 2011; Acke,
Dhooge, Malfait, & De Leenheer, 2012; Blanco-Kelly et al.,
2015; Lammens, Verhaert, & Desloovere, 2013;
Rajenderkumar, Bamiou, & Sirimanna, 2005).
The otolaryngologist will also assess for transient
conductive hearing changes secondary to amniotic fluid/
debris in the ear canal, cerumen occluding the ear canal,
or middle ear fluid. Whereas middle ear fluid may
constitute a transient condition in the infant with
typical anatomy, the possibility of long-standing,
fluctuating chronic middle ear fluid in children with cleft
palate, Down syndrome, or other craniofacial anomalies
must be considered in terms of requiring amplification and/
or visual communication due to prolonged periods of time
with compromised audibility of speech sounds (Nicholson
et al., 2011; McCreery, Walker, et al., 2015).
Radiologic imaging. Temporal bone imaging is
indicated when families seek the etiology for children who
are suspected to be deaf or hard of hearing, as it may
further characterize the etiology, identify anatomical
markers for progression, predict prognosis from
interventions such as amplification and/or cochlear
implantation, and identify lesions of the central nervous
system that require medical/surgical intervention for the
18
Table 1
Risk Factors for Early Childhood Hearing Loss: Guidelines for Infants who Pass the Newborn Hearing Screen

Perinatal

Note. AAP = American Academy of Pediatrics; ABR = auditory brainstem response; AABR = automated auditory
brainstem response.
* Infants at increased risk of delayed onset or progressive hearing loss
**Infants with toxic levels or with a known genetic susceptibility remain at risk
***Syndromes (Van Camp & Smith, 2016)
****Parental/caregiver concern should always prompt further evaluation.

overall health of the patient (Lowe & Vézina, 2005).
Malformations of the external, middle, and inner ear as
well as the internal auditory canal are clearly detectable
using currently available imaging. Structural anomalies of
the cochleovestibular nerves and brain are also
discernable in most cases. In general, high resolution
computed tomography (HRCT) is well-suited for assessing
the osseous structures (external auditory canal and middle
ear), while magnetic resonance imaging (MRI) provides
excellent soft tissue and fluid detail for looking at the
cranial nerves and brain (Lowe & Vézina, 2005). The inner
ear including the vestibular aqueduct (endolymphatic duct)
is well visualized using either MRI or HRCT.

The Journal of Early Hearing Detection and Intervention 2019; 4(2) 19

For cases of aural atresia and other conductive
conditions, HRCT remains superior to MRI for assessing
bony detail. For children with newly identified sensorineural
or mixed types of hearing loss, MRI allows direct imaging
of the inner ear, cochlear nerves, and brain without the
need for cranial irradiation. These imaging studies may
also be used to assess potential candidacy and prognosis
for surgical intervention, including reconstruction, bone
conduction hearing devices or implants, and cochlear
implantation. For cochlear implantation, MRI is usually
sufficient except in select situations where facial nerve
anomalies, inner ear ossification (Adunka, Jewells, &
Buchman, 2007), patency of the bony cochlear nerve
canal (Adunka et al., 2007), and temporal bone pathology
(Roche et al., 2010) are in question. The finding of
cochlear nerve deficiency or severe inner ear
malformations on imaging may directly affect the prognosis
for the development of open-set speech perception and/or
spoken language using amplification or cochlear implants
(Young, Ryan, & Young, 2014).
Additional testing considerations. Additional
laboratory testing to be considered in the process of
evaluating a newborn or infant diagnosed with
sensorineural hearing loss and to rule out the most
common etiologies that impact intervention plans include
(a) connexin gene, (b) electrocardiogram, and (c) urine
and saliva swabs for congenital cytomegalovirus (cCMV).
While connexin and electrocardiogram tests are briefly
mentioned here, due to their importance they are covered
in more depth in the next section regarding genetic
testing. Furthermore, significant evidence is presented in
the cCMV section to support JCIH recommendations.
Connexin 26 and Connexin 30 genes. Mutations in this
gene account for a significant number of non-
syndromic cases (Orzan & Muria, 2007; Tarkan et al.,
2013). As the inheritance is autosomal recessive, these
children frequently have a negative family history for other
family members who are deaf or hard of hearing. In the
absence of other risk factors, these children may or may
not pass the birth screen. Developmental surveillance
and parental concern play an important role in monitoring
infants who are unknowingly at risk for progressive hearing
changes due to this genetic condition.
Electrocardiogram. An electrocardiogram is carried out
among children who are profoundly deaf to identify a long
QT interval (Jervell and Lange-Nielsen syndrome; Tarkan
et al., 2013). This rare autosomal recessive inherited
condition can result in sudden death if undetected and
therefore, the benefit far outweighs the cost of what may
seem an unnecessary medical procedure.
Congenital Cytomegalovirus (cCMV). CMV infection is
a leading cause of congenital infection, occurring in 0.2 to
2 percent of live births worldwide and is a leading cause
of non-genetic unilateral or bilateral sensorineural hearing
loss (Cannon, Griffiths, Aston, & Rawlinson, 2014; Doutre,
Barrett, Greenlee, & White, 2016; Ross et al., 2014;
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Yamamoto et al., 2011). About 25,000 infants are born
each year in the United States with congenital CMV
infection, 10–15% of whom develop sensorineural hearing
loss (Cannon et al., 2014). Since children with cCMV can
develop late onset sensorineural hearing loss (Cannon
et al., 2014), all infants who test positive on a neonatal
screen for CMV require periodic monitoring by audiology to
identify changes in hearing thresholds, with the
provision of appropriate amplification and early
intervention as indicated.
A standardized, high-throughput test suitable for cCMV
newborn screening does not currently exist, but
development of an appropriate and relatively
inexpensive screening is a priority research topic (NIDCD,
2002). Another research priority is antiviral treatment of
newborns that have cCMV. Administration of parenteral
ganciclovir has shown protection against hearing
deterioration in some studies but not others, and as there
are concerns about toxicity, randomized control trials are
needed (Hamilton et al., 2014; Smets et al., 2006).
Role of the Geneticist and the Genetics Counselor
The medical geneticist is responsible for the interpretation
of family history data, the clinical evaluation and diagnosis
of inherited disorders, and the performance and
assessment of genetic tests. Geneticists, along with
genetic counselors, provide genetic counseling to families.
Geneticists and genetic counselors are uniquely qualified
to interpret the significance and limitations of tests and
to convey the current status of knowledge during genetic
counseling. All families of children who are confirmed as
deaf or hard of hearing should be offered a genetics
evaluation and counseling (Alford et al., 2014). This
evaluation can provide families with information on
etiology, prognosis for progression, associated disorders
(e.g., renal, vision, cardiac), and likelihood of recurrence in
future offspring (or the offspring of close relatives).
Occasionally, a genetic determination can identify a
particular syndrome that is associated with otherwise
unrecognized medical conditions that require intervention.
Incidence of genetic disorders. In large population
studies, at least fifty percent of the etiologies related to
being deaf or hard of hearing are designated as
hereditary; hundreds of syndromes and many different
individual genes have been identified (Morton & Nance,
2006; Santos et al., 2005). The evaluation, therefore,
should include a review of family history of specific
genetic disorders or syndromes, genetic testing for gene
mutations such as GJB2 (connexin-26), and syndromes
commonly associated with early-onset hearing loss (see
Risk Factors, Table 1). As the widespread use of newly
developed conjugate vaccines decreases the prevalence
of infectious etiologies such as measles, mumps, rubella,
Haemophilus Influenza Type B, and childhood meningitis,
the percentage of cases of early onset hearing loss
attributable to genetic etiologies can be expected to
increase, thereby improving the utility of the
recommendation for early genetic evaluations (Alford et al.,
20
2014). Connexin 26 and 30 genes code for the production
of gap junction proteins, which allow for ion chemical
balance (potassium) in the inner ear. Mutations in the
connexin 26 (and to a lesser degree connexin 30) genes
account for a significant number of non-syndromic cases.
The decision to obtain genetic testing is dependent on
informed family choice in conjunction with standard
confidentiality guidelines (Chu et al., 2015).
Genetic testing. Although an individual family may choose
not to participate in genetic testing for a variety of reasons,
the American College of Medical Genetics recommends of-
fering genetic counseling and genetic testing for all infants
who are deaf or hard of hearing and their families (Alford
et al., 2014). New technologies are changing the genetic
diagnostic process. Chromosome microarray technology
(looking for known micro-deletions and micro-duplications),
with an expected yield of 15–18% in a child with
malformations or developmental delay, is beginning to
replace routine karyotyping, since most of these alterations
are not visible on routine cytogenetics (Chu et al., 2015).
In the near future, multi-gene panels may replace some of
the diagnostic studies previously done on non-syndromic
patients. For example, molecular testing is available for
Usher syndrome, the long QT syndromes and the
hereditary nephropathies. The panel also includes testing
for Connexin 26 and other common gene abnormalities
producing pre-lingual and post-lingual non-syndromic
hearing loss.
Counseling about the results of genetic testing may
influence the family’s decisions regarding intervention
options for their child. A recent Australian longitudinal study
suggests that genetic testing can be valuable in
determining etiology (Dahl et al., 2013). This study
completed molecular testing of perinatal Guthrie newborn
dried blood spots of children identified as deaf or hard of
hearing either as neonates or after the newborn period
(Dahl et al., 2013). They analyzed the GJB2 and SLC26A4
genes for the presence of mutations, screened for the
mitochondrial DNA A1555G mutation, and screened for
congenital CMV infection in DNA. Results were obtained
for 364 children and etiology was established for sixty
percent of these children. One or two known GJB2
mutations were present in 82 of the children. Twenty-four
children had one or two known SLC26A4 mutations. GJB2
or SLC26A4 changes with unknown consequences on
hearing were found in 32 children. The A1555G mutation
was found in one child, and CMV infection was detected
in 28 children. Auditory neuropathy was confirmed in 26
children whose DNA evaluations were negative.
Every identified infant should have a regular evaluation by
an ophthalmologist to document visual acuity and rule out
concomitant or delayed-onset vision disorders, such as
cataracts or Usher syndrome (Dammeyer, 2012). Indicated
referrals to other medical subspecialists, including
developmental pediatricians, neurologists, cardiologists,
and nephrologists, should be facilitated and coordinated by
the PCP.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Timely intervention for the infant. Ensuring that the
identified child has access to developing receptive and
expressive language is of paramount importance. Whether
that language is spoken, sign (e.g., American Sign
Language) or a combination (bilingual) is of lesser
importance than ensuring access to language and
resultant linguistic competence (Kushalnagar et al., 2010;
Poliva, 2016). The JCIH Supplement on Early Intervention
(JCIH, 2013) addresses best-practices in language
development and intervention.
The great majority of infants identified through UNHS
programs have sufficient residual hearing (levels ranging
from mild to severe) to permit benefit from hearing aid
amplification (Holte et al., 2012). Accordingly, hearing
aid amplification is the typical primary treatment for most
identified infants. That being said, it would be very much
an oversimplification to consider amplification alone as an
adequate intervention. A broader discussion of early inter-
vention guidelines was recently published as a supplemen-
tal statement by the JCIH (2013), and that supplemental
statement provides additional detail not included in the
JCIH (2007) statement.
Role of the Audiologist Following Diagnosis
It is often the audiologist who provides the first information
to the family for an identified child following diagnosis. The
audiologist has the responsibility for communicating the
information to the family in an empathetic, non-biased,
open-ended fashion, and in a language (spoken or signed)
that is accessible to the parents, using interpreters/
translators as necessary (Fitzpatrick, Durieux-Smith,
Eriks-Brophy, Olds, & Gaines, 2007; Pizur-Barnekow,
Darragh, & Johnston, 2011). Families retain information
at different rates and require different styles of information
delivery. Because of the complexity and volume of
information, redundancy in the message and delivery of
the message through multiple avenues (e.g., written,
verbal, website, video presentation, etc.) is important.
Most often, the audiologist is responsible for referral to the
early intervention system. In some systems, the
audiologist is the primary professional who conveys
information about early intervention opportunities; in other
sites it is an early intervention provider with specialized
knowledge and skills in working with children who are deaf
or hard of hearing. Early, careful, and comprehensive
education of families and caregivers regarding the nature
of language acquisition is invaluable in encouraging
families to seek appropriate early intervention services for
their child.
When counseling families, information regarding
communication modes, methodologies, and technologies
should be provided in a comprehensive and non-biased
fashion. Families should be offered written materials in an
accessible format and language. Information about
listening and spoken language, signed language, and
combined approaches should be provided. Additionally,
21
information about amplification options (hearing aids,
cochlear implants, visual and auditory assistive
technologies) should be provided as appropriate for the
infant’s audiologic diagnosis, recognizing the possibility
of progression of hearing thresholds to a more severe
degree (ASHA, 2008a). Families benefit from contact with
other parents who are trained to provide parent-to-parent
or family-to-family support, and also benefit from contact
with a trained professional who is deaf or hard of hearing
(Moeller et al., 2013).
Amplification: Hearing aids, cochlear implants, bone
conduction hearing devices, assistive hearing
technologies
Hearing aid amplification. Families should be educated
about the need for regular audiologic re-evaluation. It is
probable that the first confirmation that a child is deaf or
hard of hearing does not include, with optimal reliability
and validity, detailed information about hearing status at all
frequencies important for the development of speech and
language (Bagatto et al., 2016). Audiologic management
does not stop at the diagnosis. Regular surveillance of
hearing status is critical to ensure that hearing aid
amplification is appropriately fitted and programmed.
Progression or fluctuation of hearing thresholds can occur,
and hearing sensitivity may change or fluctuate with
concurrent episodes of middle ear effusion.
An important first step for families who have chosen
development of listening and spoken language as a goal
is the fitting of appropriate hearing aid amplification. If the
family chooses, fitting of hearing aid amplification no later
than four months of age (or as soon as there is
confirmation that the child is deaf or hard of hearing) is
optimal, if not medically contraindicated (e.g., draining ear,
local skin or ear canal condition, absent auditory nerves;
Bagatto et al., 2016).
Following diagnosis of the hearing loss, audiologists
should allow ample time to:
• listen to families and to answer their questions;
• support family decision-making;
• provide additional resources;
• provide information and referrals for family support;
• encourage families to advocate for their needs;
• use clear, simple (lay) language;
• explain the process (e.g., referral to early intervention);
• explain what will happen next (e.g., next appointment);
• explain the hearing aid or cochlear implant process,
and
• discuss visual strategies and resources.
Hearing aid selection and fitting in infants must be
conducted using evidence-based protocols and algorithms
(Bagatto et al., 2016). Consistent with pediatric diagnostic
evaluations, the fitting of pediatric amplification should
be provided by an audiologist with expertise, skills, and
knowledge in pediatric audiology (Bagatto et al., 2016).
Selection of hearing aids for infants/toddlers must include
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
features specifically designed for this age group:
pediatric-sized earhooks, tamper-proof battery doors,
and accessibility for remote-microphone (FM) technology.
Further, as progressive hearing loss is not uncommon in
young infants, hearing aids with flexible gain and output
characteristics are necessary to accommodate potential
change (decrement) in hearing in the first few months and
years of life (Bagatto et al., 2016). Many major hearing aid
manufacturers offer loaner hearing aid programs to
pediatric audiologists in an effort to minimize the time
between diagnosis and hearing aid fitting.
Some current EHDI databases do not contain
information about amplification (Uhler, Thomson, Cyr,
Gabbard, & Yoshinaga-Itano, 2014). Suggestions for
improving the standardization and reporting to state EHDI
data systems have recently been provided (Alam, 2016).
Minimally, information collected by EHDI programs should
include: (a) Whether amplification was recommended by
the audiologist, and (b) The age amplification was first
provided (either through loaner or purchase). If there is
confirmation that the child is bilaterally deaf or hard of
hearing, bilateral hearing aids should be fitted unless
medical or audiological contraindication exists or the family
does not elect to proceed.
Although toneburst ABR thresholds will most likely be used
for initial hearing aid fitting, as soon as developmentally
possible, the audiologist should use behavioral thresholds
(responses) in addition to ABR thresholds for verifying
hearing aid gain and output targets. Although ABR
thresholds are good predictors of behavioral thresholds,
established behavioral responses are the best reflection of
how the child hears (Alam, 2016). To use validated,
frequency-specific targets for gain and output, frequency-
specific thresholds (at a minimum, one low-frequency and
one high-frequency), as well as a measured estimate of
any conductive component through the use of bone-
conduction ABR or behavioral test, is necessary.
An emerging technique for hearing aid fitting and validation
is through the use of CAEP (Punch, Van Dun, King, Carter,
& Pearce, 2016). Small, Ishida, and Stapells (2017)
reported on cortical response recordings demonstrating
binaural lateralization and binaural hearing processes.
Although not yet in widespread use in the United States,
CAEPs have been shown to confirm audibility of speech
sounds at the cortical level and to objectively demonstrate
auditory discrimination. CAEP can be recorded at a very
young age and has the specific advantage of avoiding use
of sedation and anesthesia while providing validation of
hearing technology (Punch et al., 2016).
Optimization and objective re-verification of hearing aid
fitting should occur with each new earmold fitting, and as
puretone threshold information changes. Optimization
implies programming hearing aid gain and output relative
to an evidence-based, validated pediatric amplification
target (i.e., Desired Sensation Level, Bagatto et al., 2016;
or National Acoustics Laboratories, Ching et al., 2013).
Initial amplification, as well as each new earmold fitting
22
should be accompanied by (re-)measuring the child’s real
ear to coupler difference (RECD) with the earmold in place
to optimize the hearing aid settings to individual child-
specific amplification targets across the speech spectrum.
Regular audiologic evaluation is required to monitor any
fluctuation or decrement in hearing; should changes in
hearing sensitivity occur, hearing aid programming and
options may require modification to accommodate the
change in hearing. Ongoing validation of amplification
fitting is mandatory to ensure that the purpose of hearing
aids (i.e., development of spoken language, auditory
awareness, or other) is being fulfilled by the amplification
arrangement (Bagatto et al., 2016). An adequate earmold
fit is critical, and new earmolds are required as the child
grows; this is a particular concern in infants, for whom
more rapid ear growth can be anticipated.
The purpose of hearing aid amplification in infants who are
deaf or hard of hearing is to facilitate timely and optimal
auditory development as a precursor to development of
spoken language (Meinzen-Derr, Wiley, Creighton, &
Choo, 2007; Wiley & Meinzen-Derr, 2013). The
expectations for timely progress in acquiring spoken
language, of course, must be individualized, based on the
nature (type) and degree of hearing loss, the child’s other
special needs (if any), the early intervention program, and
the level of family involvement. Progress in developing
effective communication using listening and spoken
language must be regularly assessed. If the infant/child is
not meeting expected language milestones with
appropriately fitted hearing aid amplification alone,
additional language and communication approaches andor
technologies should be considered. Approaches might
include ASL, cochlear implantation, or additional hearing-
assistance technologies (Wiley & Meinzen-Derr, 2013).
Assistive technologies. Assistive technologies
encompass a variety of devices, both auditory and
visual as well as vibrotactile. Technologies include listening
devices beyond the hearing aid, such as wireless
transmission from a remote microphone, visual
communication technologies such as CART
(communication access realtime translation), Skype or
computer-based video transmission, devices to amplify
telephone communications, and devices to provide visual
translation of auditory stimuli in the home such as flashing
doorbells. Remote-microphone technology is optimal in
situations involving noise and distance. Once a child be-
gins to spend more time at distances away from the
person speaking or in noisy situations (e.g., car), the use
of hearing assistive technologies as well as visual and
tactile assistive technologies should be considered.
Bone conduction hearing device/implant
considerations, requirements, expectations. Continuing
audiological and medical surveillance as well as provision
of information and education and support for the family
is necessary to optimize development and treatment for
the infants and toddlers who are deaf or hard of hearing.
Medical management varies, ranging from the removal of
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
cerumen from the ear canal, to the treatment of concurrent
middle ear effusion or infection, to long-term plans for
reconstructive surgery and assessment of candidacy
for cochlear implants. If necessary, surgical treatment of
malformation of the outer and middle ears (including bone
conduction hearing devices) should be considered in the
intervention plan for infants and toddlers who are deaf or
hard of hearing with permanent conductive or mixed
hearing thresholds when a child reaches an appropriate
age (Whitton & Polley, 2011). If surgical treatment is
planned, evaluation of hearing needs as well as cosmetic
gains must both be considered. Infants and young children
can be fit with and wear bone conduction hearing devices
on a softband until they are considered potential implant
candidates at age five. Surgical treatment that is designed
to improve cosmetic appearance of the external ear may
impede successful use of hearing aids as a result of
changes in the pinna or ear canal.
Cochlear implantation considerations, requirements,
expectations. If the parents’ goals for their child include
development of spoken language, cochlear implants are
the mainstay of treatment for most children who are deaf
with thresholds in the severe to profound range. Cochlear
implants are indicated for children (> 12 months of age)
with bilateral severe-to-profound sensorineural hearing
loss (including auditory neuropathy) who fail to make
expected progress with appropriately fitted amplification.
Comprehensive pediatric hearing centers integrate the
assessment and intervention phases for children who are
deaf, making hearing aid and cochlear implant transitions
seamless. Minimally, audiologists and PCPs must work
together with the parents and early intervention specialists
to recognize implant candidates and make prompt
referrals.
At the present time, there is no single comprehensive
best-practices document regarding pediatric cochlear
implantation. Cochlear implant surgery around 12 months
of age or younger offers the greatest chance of significant
open-set speech understanding with resulting language
acquisition rates that match those of normal hearing peers.
Timing of the intervention remains critical, with better
outcomes achieved for those receiving an implant by two
years of age (Ching et al., 2009; Dettman, Pinder, Briggs,
Dowell, & Leigh, 2007). Studies have documented the
critical nature of early implantation for the development of
spoken language (Niparko et al., 2010). It is noteworthy
that nearly all children with no responses to multi-
frequency toneburst ABRs are audiologically cochlear
implant candidates (Hang et al., 2015). With this in mind,
these children should be considered for fast-tracking
surgery in the first year of life to avoid unnecessary delays.
Cochlear implants can be provided unilaterally or
bilaterally (which may be simultaneous or sequential),
and can be used in combination with amplification (i.e.,
bi-modal/hearing aid in one ear and cochlear implant in the
other) [Ching & Dillon, 2013; Scherf et al., 2009a, 2009b).
23
Binaural (i.e., two ear) hearing improves sound localization
and hearing in noise. For children, the binaural benefit
presumably can enhance incidental learning, an important
skill for language development. Although there still remains
some controversy regarding the relative benefits of
bimodal versus bilateral implantation, it is clear that the
binaural benefit is best achieved for children receiving
two implants (Steel, Papsin, & Gordon, 2015). Moreover,
bilateral implants offer a built-in backup system to avoid
complete loss of sound if one device fails or is lost or
broken. Hybrid cochlear implants that combine electrical
and acoustic stimulation of the auditory system in the
same ear are FDA-approved for use in adults, but have yet
to be tested or approved for use in infants or children. It is
probable that this emerging and potentially-beneficial
technology will become available and approved for
children in the future (Gantz et al., 2010).
The decision as to whether a child is a cochlear implant
candidate begins with the audiological and medical
assessment. For children who are appropriate candidates,
the decision to pursue implantation is then the choice of
the family, based on their communication desires for their
child. The family must also have reasonable expectations
and a clear understanding of the continued need for
intensive, auditory-based intervention and audiological
management (Weisel, Most, & Michael, 2007).
Outcomes following cochlear implantation can be impacted
by a number of variables that include: age at implantation
(Ching et al., 2009; Dettman et al., 2007; Niparko et al.,
2010), progression of hearing loss (Barreira-Nielsen et al.,
2016; Fitzpatrick, Ham, & Whittingham, 2015), duration of
device use, cochlear morphology and cranial nerve VIII
integrity as determined on imaging (Buchman et al., 2011),
precise electrode placement in the scala tympani,
presence of additional neurodevelopmental challenges,
high quality mapping of the speech processor, parental
educational level, and involvement in family centered,
intensive auditory-based intervention (Iseli & Buchman,
2015).
Referral to early intervention services. The purpose of
early intervention is to achieve optimal child and family
outcomes. Hence, the audiologist must make the referral
for Part C Early Intervention (IDEA, 2004) services as
quickly as possible following confirmation that a child is
deaf or hard of hearing. Federal regulations require that
this referral be made within seven working days of
diagnosis (IDEA, 2004). In cases of congenital aural
atresia, the referral can and should be made by the birth
hospital. Despite these requirements, 2013 EHDI statistics
from the CDC (n.d.-a, n.d.-b) indicate that only 63.9% of
the children identified as deaf or hard of hearing are
currently enrolled in early intervention services (Alam,
2016; Uhler et al., 2014).
Referral to early intervention should not be deferred until
audiologic diagnostic evaluation and hearing aid fitting are
completed. For all infants who are confirmed as being deaf
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
or hard of hearing, either unilateral or permanent bilateral
in type, a referral to early intervention must be made.
Reports should also go to the infant’s medical home,
primary care provider, pediatric otologic physician (if
known), and to the state EHDI program. The infant’s
primary care provider should be kept apprised of all
findings as evaluations and early interventions continue.
Early Intervention: Services for
Infants/Toddlers from Birth to Age Three
Years and their Families
Some states currently characterize their birth-to-three
programs as early development programs rather than
early intervention programs. For the 2019 statement, the
JCIH made a decision to retain the term early intervention,
because it is referenced in federal law and is familiar to
readers. However, it is suggested that future
consideration be given to using a term like early
development that captures the proactive and capacity-
building nature of the intervention with identified infants,
toddlers, and young children and their families.
There is growing evidence that infants who are identified
early in life and provided with timely early interventions
demonstrate better linguistic outcomes than later-identified
infants and children. These effects have been observed in
vocabulary development (Mayne, Yoshinaga-Itano, Sedey,
& Carey, 1998), receptive language (Kennedy et al., 2006;
Vohr et al., 2012; Watkin et al., 2007; Yoshinaga-Itano
et al., 2010), expressive language (Pipp-Siegel, Sedey,
VanLeeuwen, & Yoshinaga-Itano, 2003; Yoshinaga-Itano
et al., 2010), syntax (Yoshinaga-Itano et al., 2010), speech
production (Ambrose, Unflat Berry, et al., 2014;
Yoshinaga-Itano, Coulter, & Thomson, 2000) and social-
emotional development (Pipp-Siegel, Sedey, & Yoshinaga-
Itano, 2002; Yoshinaga-Itano, Sedey, Coulter, & Mehl,
1998). Tomblin and colleagues (Tomblin et al., 2014;
Tomblin et al., 2015) demonstrated a positive impact on
longitudinal language growth trajectories when
amplification was provided early for children who are hard
of hearing.
Selected population-based studies from the past 10 years
did not find an association between age of identification
and later child outcomes (Fitzpatrick et al., 2007; Geers
et al., 2009). In part, these differing results may reflect the
fact that the distributions for age of identification are quite
distinct for groups studied prior to and after the
implementation of newborn hearing screening (McCreery,
Walker, et al., 2015). In addition, the reasons for late
identification may differ in the pre- and post-newborn
hearing screening contexts, which further impacts the
respective study populations (McCreery, Walker, et al.,
2015). Additionally, for deaf children with cochlear
implants, age at implantation has been found to be a
stronger predictor of spoken language outcomes than age
at identification (Geers et al., 2009; Ching, Leigh, & Dillon,
2013).
24
Providing children and families the earliest possible start in
intervention is considered best practice (Joint Committee
on Infant Hearing, 2013; Moeller et al., 2013). Early
interventions are widely recognized as a means to provide
infants access to linguistic input, whether through spoken
and/or sign language. Access to linguistic input is regarded
as essential for successful communication and language
development. Data from hearing children show that simply
being exposed to more words is a very potent factor
driving communication development (Hart & Risley, 1995;
Hurtado, 2009; Suskind et al., 2013). This practice of
providing early access is supported by recent longitudinal
studies, showing the positive influence of early ages at
device fitting and duration of device use on children’s
outcomes (Nicholas & Geers, 2007; Sininger et al., 2010;
Tomblin et al., 2014, 2015). In spite of improving outcomes
for children who are deaf or hard of hearing, selected
epidemiological studies suggest that, on average, some
children remain at risk for delays in spoken language, even
when identification is early (Tomblin et al., 2015; Ching et
al., 2013).
Early interventions are designed to minimize or prevent
such delays and to promote linguistic development and
access through high quality communicative interactions
in the home. To accomplish this, early interventions are
designed to promote caregiver use of language facilitation
strategies. Several studies indicate a linkage between
parental communication and child language outcomes. For
example, large-scale investigations of parent-child
interactions demonstrated the positive influence of
caregiver use of language-promoting strategies on child
language outcomes (Cruz, Quittner, Marker, & DesJardin,
2013; Quittner et al., 2013) and the importance of
avoidance of overly directive interactions (Szagun &
Schramm, 2016; Ambrose, Vandam, & Moeller, 2014).
Szagun and Schramm documented the strong influence of
the home linguistic environment on development in young
children who are deaf. Child language outcomes were
also found to be enhanced when caregivers engaged their
toddlers who were hard of hearing in frequent
conversational turns (Ambrose, Vandam, & Moeller, 2014;
Hoffman, Quittner, & Cejas, 2015). Hoffman and
colleagues found exposing deaf children early to language
has a significant and positive impact on children’s social
competence.
Several family and child background factors have been
linked with child outcomes, including absence of additional
disabilities, severity of hearing thresholds, gender, and
maternal education and age at activation of cochlear
implants (Geers et al., 2009). Higher levels of family
involvement in promoting children’s communication are
associated with enhanced child language outcomes
(Watkin et al., 2007). Greater maternal stress was
associated with greater risk for vocabulary delays and for
child behavioral challenges (Topol et al., 2011). Although
more research is needed, enhanced maternal self-
efficacy may be a particularly beneficial family outcome
from early intervention (DesJardin & Eisenberg, 2007;
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Stika et al, 2015). Collectively, these results support the
value of providing early access to services to ensure that
language and communication development is optimized for
the child and family.
It is widely recognized that a key component of providing
quality services is the expertise of the provider,
including knowledge and skills that are specific to working
with children who are deaf or hard of hearing and their
families (JCIH, 2013; Moeller et al., 2013). These services
may be provided in the home or in a center, in a
combination of the two locations, or in community-based
environments. However, home-based services were found
to be associated with higher levels of family participation
than non-home-based locations (Harrison et al., 2016).
Family-Centered Practices
The Early Childhood Technical Assistance Center (ECTAC;
2016) describes family-centered principles as a set of
interconnected beliefs and attitudes that shape directions
of program philosophy and behavior of personnel as they
organize and deliver services to children and families. In
2014, the Division for Early Childhood, Council for
Exceptional Children published recommended practices
for early intervention. Sensitivity and respect for the culture
and values of individual family members and each family’s
ecology is the core of these services, as members define
the people, activities, and beliefs important to them. The
definitions of family-centered care from different fields
and disciplines often include these common descriptors:
strengths-based, consumer-driven, family systems, family
support, empowerment, proactive service delivery,
competency-focused, partnerships, collaborative
relationships, and family-driven (Dunst, Trivette, & Hamby,
2007; Dunst & Dempsey, 2007).
All of these general principles apply when the family has
an infant/toddler who is deaf or hard of hearing. As
partners, the family and professionals often place primary
emphasis on communication and language access for the
infant/toddler, and on provision of a language rich
environment throughout daily routines (Quittner et al.,
2013; DesJardin, Ambrose, & Eisenberg, 2009). A
primary goal of the early intervention program is to foster
families’ abilities to provide an abundance of age-
appropriate language opportunities, joint engagement
routines, and regular conversational turns throughout
natural daily interactions. Families are supported in
developing the confidence and competence to promote
the infant/toddler’s development through attunement to the
child, providing an engaging social-emotional climate, and
by creating and maximizing natural language and other
learning opportunities. Families gain an understanding that
their efforts to stimulate language development and
provide language access in a time-sensitive manner are
critical steps in addressing the needs of the child to
optimize social, cognitive, and linguistic outcomes. The
role of the professional in this enterprise is that of
supporter, partner, and coach (Rush & Shelden, 2011).
25
Goals for Early Intervention Programs
In 2013, the JCIH published a supplement to its 2007
Position Statement, entitled, “Principles and Guidelines for
Early Intervention Following Confirmation That a Child Is
Deaf or Hard of Hearing.” The goals listed with details
regarding the rationale, guidelines, and benchmarks for
each can be found in the complete document. The
supplement acknowledges that early intervention is a
primary goal of the EHDI process. Best practices dictate
the need for evidence-based, carefully designed, and
individualized intervention, and for early education
programs that are implemented promptly, using service
providers with optimal knowledge and skill levels.
At the current time, there are major gaps in the evidence
about the ways in which service provision interacts with
child and family characteristics to influence outcomes
(Eisenberg et al., 2007). In spite of limitations and gaps in
the evidence, the literature does contain research
studies in which all identified children had access to
similar, well-defined early intervention services. These
studies indicate that positive outcomes are possible, and
they provide guidance about key program components that
appear to promote these outcomes. The JCIH 2013
supplemental statement on early intervention was
drafted by a team of professionals with extensive expertise
in early intervention programs for children who are deaf or
hard of hearing and their families. These early intervention
experts relied on extensive literature searches and review
of current studies, existing systematic reviews, and recent
professional consensus statements in developing goals
listed below and related best practice guidance (Centre for
Allied Health, 2008). Twelve best practice goals for early
intervention may be found in the JCIH 2013 supplement on
early intervention.
Supporting families in the process of informed
decision-making. Families of infants and toddlers who
are deaf or hard of hearing encounter new concepts and
technical information very early in the intervention process,
and they often need to make a number of decisions (e.g.,
about amplification, languages, and modalities) leading to
communicative competence at key stages of the child’s
development. Informed decision-making is a fluid and
ongoing process that requires a family to consider new
knowledge, information, experiences, language
assessment results, and their personal family goals and
values as they make decisions. Service providers must
strive to collaborate and work together with families on the
process of gaining the necessary knowledge, information,
clarity, and experiences that support fully-informed
decisions. Elements that impact a family’s decisions
include their child’s unique strengths and abilities, their
family’s value and belief system, social circumstances,
cultural influences, and personal comfort level in making
choices on behalf of the child.
Young and colleagues (2006) emphasize that informed
choice is not synonymous with the provision of information
that is neutral or functionally descriptive. Rather,
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
evaluative information is essential in that it draws attention
to the various benefits, risks, and family responsibilities
that are associated with particular decisions and/or
choices. This suggests that it is incumbent upon
professionals and family support programs to provide
comprehensive guidance about expected child outcomes
as well as what the family can expect to commit to when
selecting specific intervention approaches or devices.
Ultimately, decision-making authority rests with the family
(Dunst, Trivette, & Deal, 1988). Collaborations with
professionals support families’ abilities to exercise this
authority. The Division for Early Childhood of the Council
for Exceptional Children (2015) published checklists to
assist early intervention providers in assessment of
services for family centeredness, family engagement,
informed decision-making, and family capacity building.
Each family should be fully informed about various
approach(es) to language and communicative
development, and should be fully informed of the
necessity for promotion of language(s) acquisition at the
earliest possible time following identification. Families
should be encouraged to consult with their early
intervention team, other specialists (e.g., audiologists,
speech-language pathologists, ASL or other sign language
specialists, adults who are deaf or hard of hearing,
physicians), their child’s health care provider, and
other parents of children who are deaf or hard of hearing
throughout the decision-making process. It is beyond the
scope of the current JCIH document to provide
comprehensive coverage of the various options to promote
language access. Readers are referred to the 2013
Supplement to the JCIH 2007 document for further
guidance.
Supporting family and infant mental health. Early
intervention providers work with a diverse array of families
who have a range of individual needs, circumstances, and
resources. The early identification period of learning,
gathering resources, and making decisions is naturally
stressful for families. However, concerns arise if elevated
life stressors persist at a level that creates parenting
challenges that may impede natural bonding and
acceptance of the child being deaf or hard of hearing.
Previous studies find that, on average, hearing parents
of children who are deaf or hard of hearing do not report
elevated levels of stress in general aspects of parenting
(Åsberg, Vogel, & Bowers, 2007; Dirks, Uilenburg, &
Rieffe, 2016; Meadow-Orlans, 1994). However, some
families may be at risk for context-specific stressors
related to the child being deaf or hard of hearing. These
may include communication challenges and educational
concerns, behavioral issues, maintaining use of visual
and auditory devices, receiving several different types of
services, and ensuring that family members and teachers
are communicating effectively with the child using visual
and/or spoken language (Hintermair, 2006). High levels of
parenting stress may affect outcomes for children who are
deaf and parental access to personal and social resources
reduces stress (Hintermair, 2006).
26
For some families, especially those living with extreme
stressors (e.g., poverty, depression), mental health
concerns have the potential to disrupt healthy mother and
infant or child attachment and interventions focused on
maternal sensitivity may be advisable. Maternal stress
and depression have been linked to levels of mother-child
attachment security (Atkinson et al., 2000). Infant Mental
Health specialists and Home Visiting programs (Filene,
Kaminski, & Valle, 2013) may be a useful resource to
EHDI teams when families are dealing with significant and/
or lingering and unresolved life stressors. See the World
Association for Infant Mental Health (http://waimh.org) and
the HomVEE (https://homvee.acf.hhs.gov/) websites for
more information.
EHDI programs need to ensure that individualized family
supports are accessible (Jackson, 2011), and that teams of
deaf, hard of hearing, and hearing EI providers have
training to promote parental sensitivity and adjustment
while preparing families to develop positive self-concept in
the child who is deaf or hard of hearing. These supporting
factors can empower families to learn more about being
deaf and begin to make key decisions and take actions
that will fully support the child’s self-concept and learning.
Ongoing surveillance and further research are needed
related to supporting family and infant mental health.
Supporting a new generation of families. Modern
families take on many forms and may involve multiple
generations. Early intervention programs must be flexible
in identifying ways to involve family members and support
systems that play key roles in the infant and young child’s
life. Today’s generation of families tend to access
information through the on-line resources, including both
from trustworthy resources or potentially unreliable
sources of information. They may engage in opinion shar-
ing through social media. Early intervention programs need
to identify and respond to families’ preferred modes for ac-
cessing information, guiding them toward reliable sources
and networking with one another. To support family mem-
bers in gathering information to make informed decisions,
attention must be given to adult learning theories,
recognizing that adults do not learn the same way that
children learn. Professionals must become familiar with
basic theories and principles of adult learning and with
learning styles present among multigenerational dynamics
(Kuhn & Pease, 2006).
Supporting families during service transitions. As more
children who are deaf or hard of hearing are identified
early in life, service transitions may be happening at
different times based on the quality of the early services
provided, the progress of the individual child, and family
circumstances (Walker et al., 2014). The most common
transition following early intervention birth-to-three services
happens when the child turns three years of age and
services are then transitioned from IDEA Part C (early
intervention support services) to Part B (school-aged)
services, or to the education system in their geographic
region (U.S. Department of Education, n.d.-a). In general,
the transitional support services provided by professionals
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
should be smooth, informative to caregivers, family-
centered, child-focused, and assessment-based. Bridge to
Preschool is a publication modeling an effective
process for helping families through this transition
(Colorado School for the Deaf and the Blind, 2011).
Resources for supporting transitions from Part C to Part
B are also available from the Early Childhood Technical
Assistance Center website (ECTAC, n.d.).
Smooth transitions are best supported by the professional
who knows the family well, with the processes beginning
well ahead of the anticipated transition date. According to
federal law, a transition must begin by at least six months
prior to the child’s third birthday. Hands & Voices, a
national parent driven organization dedicated to supporting
families of children who are deaf or hard of hearing, has
recommended that several key topics should be discussed
during home visits as early as twelve months before the
child’s third birthday (Johnson, 2001). Such topics may
include educational placements and timelines,
identifying possible preschools, discussing the child’s
learning styles and mode(s) of communication with regard
to group settings, meeting other families of preschoolers,
and other pertinent family-identified questions. The family-
infant specialist/home visit provider is best equipped to
provide these initial discussions as they are likely to be the
professionals who know the family best and can provide
initial professional opinions on potential preschool
placements. A specific transition plan that considers
eligibility for services and Individual Family Services
Plan (IFSP) and Individualized Education Program (IEP)
differences should be completed prior to the third birthday.
The IEP and transition portfolio should be completed on or
before the child’s third birthday. If a child has equipment
(including hearing aids, cochlear implants, visual
technology and/or FM systems) to support his or her
learning or communication, the proper use of that
equipment should be documented and monitored as part
of the preschool transition plan to ensure that the child
can continue to build language and learn in his or her new
setting.
A transition that is both family centered and informative to
families carefully considers the family’s observations and
goals for their child, and also provides the family with
information to make sound decisions about transition.
Families must understand placement opportunities and
the ways in which they address the communication access
needs of the child, assessment information, what to expect
in the next phase, recommended opportunities for
services, and the differences between an IFSP and an IEP.
It is important to ensure that the needs of children who
transition with age-appropriate language and
communicative skills continue to be monitored and
supported. Children who are maintaining their trajectory of
developmental growth through preschool will have at
minimum: proper hearing assistance technology and/or
visual technology use; articulation support for spoken
language users who qualify; appropriate interpretation
services and/or direct instruction in sign language (e.g.,
ASL for ASL users); family education and resources, such
27
as Deaf mentors, ASL for families; quarterly
educational audiology support services; language and
academic growth monitoring at least every six months; and
mainstream itinerant observation or itinerant child/teacher
support as part of the preschool transition plan.
Supporting families through innovative models of
service delivery: Tele-practice. Tele-practice can be
broadly defined as the use of technology to deliver
professional services such as parent education,
assessment, and intervention over a geographical distance
(Behl & Kahn, 2015). Families who live far from qualified
providers, or who do not have the means to travel, may
encounter challenges in accessing selected specialized
early intervention services. Modern telecommunication
technology has the potential to overcome those obstacles,
but further research is needed. Although tele-practice
programs have emerged nationally and internationally,
there is a limited body of peer-reviewed empirical evidence
in the context of early intervention for families of children
who are deaf or hard of hearing. Some evidence exists in
relation to Audiological and CI practices, although most
of this work has focused on adults. Swanepoel and Hall
(Swanepoel & Hall, 2010) conducted a systematic review
of 26 peer-reviewed empirical studies of tele-practice
applications in Audiology. They concluded that initial
reports are promising, but that studies are limited in
breadth and depth, suggesting a pressing need for more
research to overcome barriers to practice application. A
few studies have empirically evaluated applications of
tele-practice for CI service delivery (Hughes et al., 2012;
McElveen et al., 2010). However, studies of the feasibility
of tele-practice for CI interventions with young children are
lacking. A recent survey (Behl & Kahn, 2015) suggests that
practitioners are using tele-practice to supplement face-to-
face services, and that there is a need to address
challenges in internet connectivity and professionals’
training in delivery of tele-practice services for early
intervention. Tele-practice applications may be helpful for
connecting families with needed supports, such as ASL
training, coaching to promote spoken language develop-
ment, and family to family connections.
Language Assessment and Intervention
Being deaf or hard of hearing occurs in people of all
nationalities, all cultural heritages, and from all language
backgrounds (Crowe, Mcleod, & Ching, 2012). This
cultural and linguistic diversity is often attributed to the
family and community in which they live (Crowe et al.,
2012). Recognition of the cultural and linguistic diversity of
young children who are deaf or hard of hearing is critical to
planning and providing assessment, habilitation, and
education services to both children and their families
(Crowe, McKinnon, McLeod, & Ching, 2013). In the
ever-increasing diversity of linguistic environments of
today’s society, the language of the home must be
taken into consideration and viewed as the foundation
from which to build.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
The process of language development includes learning
the precursors of language, such as the rules pertaining
to selective attention and turn taking (Gerken & Aslin,
2005). Cognitive, social, and emotional development are
influenced by the acquisition of language (Landry, Smith, &
Swank, 2006). Development in these areas is synergistic.
A complete language evaluation should be performed at
regular intervals for infants and toddlers who are deaf or
hard of hearing. Assessment conducted by a provider who
is fluent in the language of the home is known to increase
the reliability and validity of the assessment (Caesar &
Kohler, 2007). The evaluation should be done by qualified
professionals and include an assessment of spoken or
signed language development in the child’s dominant
language (as chosen by the parents), as well as an
assessment of the observable abilities of the child.
A primary focus of Part C services is to support families in
fostering the communication abilities of their infants and
toddlers who are deaf or hard of hearing. Spoken and/
or sign language developmental trajectories should be
commensurate with the child’s age and cognitive abilities,
and should include acquisition of phonologic, morphologic,
semantic, syntactic, and pragmatic skills for signed and/or
spoken language (Meinzen-Derr, Wiley, Grether, & Choo,
2011). If developmental goals are not being met, timely
adjustment in the intervention plan or communication
approach needs to be considered.
Families should be provided with information specific to
language development and access to peer and language
models. Families also benefit from family-involved
activities that facilitate language development of hearing
children and children who are deaf or hard of hearing
(JCIH, 2013; Moeller et al., 2013). Families should be
offered access to children and adults who are deaf or hard
of hearing, as role models, mentors, and as appropriate
and competent language models (Watkins, Pittman, &
Walden, 1998). Access to deaf or hard of hearing adults
can support caregivers in fostering the child’s emerging
self-esteem and identity formation. Information on spoken
language, signed language, or use of both should be
provided (JCIH, 2013; Moeller et al., 2013).
Medical Home and Ongoing Surveillance
Regardless of previous hearing-screening outcomes, all
infants with or without risk factors should receive ongoing
surveillance of communicative development beginning at 2
months of age during well-child visits in the medical home
(AAP Committee, 2017). This recommendation provides
an alternative, more inclusive strategy of surveillance of all
children within the medical home based on the pediatric
periodicity schedule (AAP Committee, 2017; AAP, 2014a).
All Infants who do not pass the speech-language portion
of a medical home global screening or for whom there is a
concern regarding hearing or language should be referred
for speech-language evaluation and audiology
28
assessment. This protocol permits the detection of
children with either missed neonatal or delayed-onset
hearing loss, irrespective of the presence or absence of a
high-risk indicator.
Ongoing and Continuous Surveillance, Screening, and
Referral of Infants and Toddlers
To this point, the 2019 JCIH Position Statement has
outlined the sequence of events for screening the hearing
of all newborns, providing prompt audiology evaluation for
those who do not pass newborn screening, and offering
timely intervention for identified infants. However, the child
who has a passing result on newborn hearing screening
may develop, or show evidence of, childhood hearing loss.
If one to two infants out of every thousand are diagnosed
as deaf or hard of hearing at birth, it is estimated that
another one to two per thousand will later be diagnosed
with permanent hearing loss (Mehra, Eavey, & Keamy,
2009). This may reflect delayed-onset hearing loss as well
as missed conductive, sensory, or neural hearing loss at
the time of newborn hearing screen. Infants with minimal/
mild hearing loss are likely to pass newborn screening.
The rate of being deaf or hard of hearing is known to
increase from approximately 1.2/1000 in newborns to
3/1000 in early school age. In a recent report (Watkin &
Baldwin, 2012), the prevalence of children confirmed as
deaf or hard of hearing by school age was 3.65/1000
compared to a neonatal yield of 1.79/1000.
For these reasons, all children should receive surveillance
of speech and language milestones and auditory
responsiveness in the medical home (AAP Committee,
2017). Continuing efforts to inform and educate primary
care providers about the importance of ongoing
surveillance and screening are encouraged. In addition,
programs and resources that inform and educate families
and caregivers about typical auditory development and
about typical spoken and/or signed language development
can result in more rapid identification of delayed-onset
or progressive hearing loss and/or fluctuating hearing
thresholds. Families can be encouraged to seek evaluation
should they have concerns about their child’s progress.
Education of the families and caregivers about typical
language development (spoken and/or signed) and how
to encourage and facilitate language growth in their child
would also foster earlier identification of delayed-onset or
progressive hearing loss, or otherwise unidentified
elevated hearing threshold levels.
Surveillance and Rescreening for Children with Risk
Factors
Risk factor information should be collected, stored, and
easily accessible in the electronic medical record, since
the presence of risk factors places the infant at increased
risk of delayed-onset hearing loss, regardless of the
newborn hearing screen results.
The majority of all infants identified as deaf or hard of
hearing will be followed closely by an audiologist and
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
otolaryngologist and receive indicated intervention and
support services. However, a significant number of children
will pass the newborn screen or rescreen who are at risk
for later-onset hearing loss. Table 1 shows a revised list of
risk factors since the 2007 JCIH statement. The
recommendations for follow-up and evaluation on this list
of risk factors pertain to infants who pass the newborn
screen or rescreen. These recommendations for follow-up
are based on the fact that standard newborn screening
procedures and protocols do not identify all children who
are deaf or hard of hearing due to missed mild or neural
hearing loss, progressive hearing loss, and delayed-onset
hearing loss (Johnson, 2005a; Walker et al., 2014; Nance,
2003). As shown in Table 1, the prior eleven risk factors
listed in JCIH 2007 are now listed as 12 separate factors
and divided into subgroups of predominantly perinatal (risk
factors 1–9) and perinatal or postnatal (risk factors 10–12).
In addition, the recommendations for follow-up and
monitoring have been modified and are described in
greater detail, and the age of audiologic diagnostic
follow-up for children with risk factors who passed
newborn hearing screening has been lowered for most risk
factors to by 9 months of age. Supporting evidence for the
revised JCIH risk factor recommendations is provided in
the following sections.
Perinatal Risk Factors. The first nine risk factors occur
solely in the perinatal period.
Risk Factor 1. A history of family members being deaf or
hard of hearing with onset in childhood, has consistently
been shown to be predictive that the diagnosis is second-
ary to a spectrum of genetic causes, and therefore stands
alone as a particularly concerning risk factor. Monitoring
continues to be based on both the etiology and the level of
family concern, with a diagnostic evaluation
recommended by 9 months of age (NIDCD, 2002; Morton
& Nance, 2006; Santos et al., 2005; Dedhia, Kitsko, Sabo,
& Chi, 2013) or earlier if parent or caregiver concern is
expressed (Dedhia et al., 2013). During the child’s
newborn period, some parents may not be aware of a
family history, as this information may be shared by
relatives only after the infant has not passed the hearing
screen or diagnostic testing.
Risk Factor 2. Infants who require care in the NICU or
special care nursery for more than five days is used as an
indicator of illness severity (JCIH, 2007). Although there
are a growing number of reports addressing NICU noise
exposure, such noise exposure has not been included as a
separate category, since it is included in the
classification of more than 5 days in the NICU (Daniell et
al., 2006; Lasky & Williams, 2009).
Risk Factor 3. Hyperbilirubinemia as a risk factor for
hearing loss is impacted by multiple factors including
illness severity, birth weight, rate of rise of bilirubin, clinical
findings, postnatal age of the infant, and gestational age,
as all premature infants have some degree of
29
hyperbilirubinemia. Management of these infants should
be based on established protocols (Morris et al., 2008).
Hyperbilirubinemia requiring exchange transfusion is the
only risk factor for which close follow-up is recommended
regardless of length of stay in the NICU (Oh et al., 2003;
Shapiro, 2003; Wickremasinghe et al., 2015). This
exception is made since some term infants requiring
exchange transfusion may be discharged before 5 days in
the NICU.
Risk Factor 4. Aminoglycoside administration of more
than 5 days is reaffirmed; administration of aminoglycoside
antibiotics for less than 5 days is considered a risk factor
only if toxic blood levels are identified or if there is a family
history of a mitochondrial genetic mutation associated with
aminoglycoside sensitivity for sensorineural hearing loss.
Several mitochondrial DNA mutations of the 12S rRNA
gene are known to be associated with an increased risk of
aminoglycoside-induced hearing loss. However,
aminoglycosides are one of the most common
medications administered in the NICU (Clark, Bloom,
Spitzer, & Gerstmann, 2006). Studies have examined the
frequency of the mitochondrial gene mutations in NICU
populations and identified a rate of approximately 1–1.8%
(Ealy, Lynch, Meyer, & Smith, 2011; Johnson, Cohen, Guo,
Schibler, & Greinwald, 2010). In the study by Johnson and
colleagues, 87% of infants admitted to the NICU received
aminoglycoside treatment for between 1 and 24 days and
of those, 0.9% had a mitochondrial mutation and received
aminoglycosides. Ten patients did not pass the repeat
ABR, but only a single infant with a mitochondrial mutation
that received gentamicin developed hearing loss, and this
child was extremely low birth weight. Since three children
with the mutation did not develop decreased hearing, the
authors speculate on the potential existence of a modifier
gene that conferred a protective effect. Nevertheless, low
birth weight was the primary risk factor for atypical hearing
thresholds in this study, and the duration of gentamicin
exposure for NICU infants with atypical hearing
thresholds was 5.44 days as compared to 3.92 days
for those with hearing within the typical range. Ealy and
colleagues genotyped NICU patients and adults from the
general population and identified that 1.8% of both
populations had one of the mitochondrial DNA variants.
They identified no association between the mitochondrial
variant and atypical hearing thresholds in the NICU
population.
Since approximately 400,000 infants per year are cared
for in a NICU in the United States and the majority have at
least 1 day of aminoglycoside exposure, follow-up
diagnostic audiology assessments on all of these infants is
not indicated. Based on these findings, it is recommended
that infants who received care in the NICU, who passed
the newborn hearing screen, and who received
aminoglycosides for less than or equal to 5 days, receive
standard surveillance in the medical home of hearing and
spoken language milestones following the
recommendations of the American Academy of Pediatrics
periodicity schedule (AAP Committee, 2017). The
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
recommendations may change as multi-gene panels
become standard in newborn screening. There are no
clinical indications to delay screening for eligible infants
who have had aminoglycosides administered, including
those infants who received 5 days or less, infants who
received more than 5 days, and infants who may continue
on aminoglycosides at the time of discharge.
Risk Factor 5. Perinatal asphyxia, also termed hypoxic
ischemic encephalopathy, particularly if requiring
hypothermia treatment, is noted, because of the illness
severity of this sub-group of infants and the significant
increase in permanently elevated hearing thresholds
(Shankaran et al., 2012).
Risk Factor 6. Extracorporeal membrane oxygenation
(ECMO) is specifically noted because of the increased risk
of delayed-onset hearing loss (Lasky, Wiorek, & Becker,
1998; Shankaran et al., 2012; Robertson, 1995).
Risk Factor 7. In-utero infections (i.e., herpes, rubel-
la, syphilis, and toxoplasmosis) pose a risk and require
follow-up by 9 months of age. cCMV infection is a leading
cause of congenital infection occurring in 0.2 to 2% of live
births world-wide and is a leading cause of non-genetic
unilateral or bilateral early, progressive, and delayed onset
sensorineural hearing loss (Harrison et al., 2016; Dunst et
al., 2007; Dunst & Dempsey, 2007). Therefore the
recommendation for follow-up audiologic assessment of
infants with cCMV is no later than 3 months of age. Zika
virus has been added to the list of in-utero infections
associated with hearing loss. In 2016 the CDC (2016a)
issued a series of interim guidance statements for the
caring of infants with possible congenital Zika virus
infection which included recommendations for hearing
assessment (Staples et al., 2016; Fleming-Dutra et al.,
2016; Russell et al., 2016; Leal, Muniz, Ferreira, et al.,
2016; Leal, Muñiz, Caldas Neto, et al., 2016). In October
2017 the interim guidance was updated in recognition of
the absence of data suggesting delayed-onset hearing
loss following congenital Zika virus infection
(Adebanjo et al., 2017). The updated guidance suggests
infants born to mothers with possible Zika virus exposure
during pregnancy or with findings consistent with
congenital Zika syndrome should receive a standard
newborn screen at birth, preferably using an ABR
methodology (Adebanjo et al., 2017). These infants should
be referred for automated ABR by age 1 month if the
newborn hearing screen was passed using only OAE
methodology.
Risk Factor 8. All craniofacial conditions and physical
conditions associated with hearing loss are now included
as the 8th risk factor (formerly risk factors 5 and 6).
Risk Factor 9. More than 400 syndromes and genetic
disorders associated with atypical hearing thresholds
(formerly risk factors 7 and 8) are now included as the 9th
risk factor (Morton & Nance, 2006; Santos et al., 2005; Van
Camp & Smith, 2017).
30
Perinatal or Postnatal Risk Factors. The remainder of
the risk factors may occur either in the perinatal or postna-
tal period.
Risk Factor 10. Perinatal and postnatal confirmed bacteri-
al and/or viral meningitis or encephalitis (especially herpes
viruses and varicella and hemophilus influenza and
pneumococcal meningitis are included in the 10th risk
factor (Rubin & Papsin, 2010).
Risk Factor 11. Predominantly postnatal events of
significant head trauma particularly injury to the mastoid
(Bergemalm, 2003; Zimmerman, Ganzel, Windmill, Nazar,
& Phillips, 1993) and chemotherapy (Gruss, Handzel,
Ingber, & Beiser, 2012) comprise the 11th risk factor
category.
Risk Factor 12. Family/caregiver concern regarding
hearing, speech, language, or development requires
attention. The recommendation is immediate referral
after discussion with family. One of the most important
risk factors is family/caregiver concern regarding hearing.
When a caregiver expresses concern that the baby is not
responsive to sound in the home environment, or about
speech and/or language delays, it is recommended that
the primary care provider obtain additional medical and
language history and evaluate the child carefully for middle
ear effusion. Referral to a pediatric audiologist to evaluate
hearing is recommended regardless of risk factors and
possible presence of middle ear effusion.
When risk factors for delayed-onset or progressive hearing
loss are present, comprehensive audiologic evaluation
should occur (depending on the risk factors) in the period
between hospital discharge and 9 months of age. The
schedule for on-going re-evaluation is predicated upon
both the specific risk factors and the observations by the
family of their child’s auditory and speech/language
development. In addition, for hospital readmissions in the
first month of life when there are conditions associated
with potential elevated hearing thresholds (e.g.,
hyperbilirubinemia or culture-positive sepsis), an AABR
screening should be performed before discharge even if
the baby has passed newborn hearing screening prior to
the development of the condition requiring re-admission.
Surveillance and Periodic Rescreening of Hearing in
the Early Childhood Years
Careful surveillance of hearing throughout the early
childhood years up until and including kindergarten entry
(continuous childhood screening), even in the absence of
known risk factors for hearing loss is recommended, since
the prevalence may double by school-age (NIDCD, 2005;
Niskar et al., 1998). Hearing screening may be achieved
through OAE screening in the medical home or other
pre-school settings up to age three, and use of pure tone
audiometry screening in the medical home along with a
developmental checklist for speech and language
milestone assessment is appropriate throughout early
childhood.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
These screening options may be provided by the child’s
medical home provider during regularly-scheduled
well-child visits (AAP Committee, 2017). There should be
a designated pathway for referral to a pediatric audiologist
when concerns regarding hearing and/or speech-language
development arise. Hearing screens or language-
developmental checklists may also be provided through
infant and preschool programs.
Consideration of Universal Preschool Hearing
Screening
There is a growing body of research that is beginning to
better define the prevalence of delayed-onset hearing loss
in the preschool population. Bhatia, Mintz, Hecht,
Deavenport, and Kuo (2013) reported results of a mass
screening program of 1,965 toddlers who were seen in
participating medical clinics. In an OAE screening program
offered to this population of children ranging between zero
and three years of age, the authors were able to identify
5/1965 patients (0.25%) with permanent sensorineural
hearing loss. Eiserman and colleagues (2008) also
published results from OAE screening tests performed on
children in a preschool setting. In this cohort, they were
able to diagnose 7/4519 children (0.15%) who were deaf
or hard of hearing. In a 2007 study published in Early
Childhood Research Quarterly, Eiserman and colleagues
reported on an OAE screening program of combined
pre-school testing efforts. Overall, 6/3486 children (0.17%)
were eventually diagnosed as deaf or hard of hearing.
Even if a few of the identified children in these three
studies might have actually represented children missed
at birth, it appears that the prevalence of delayed-onset
hearing loss during preschool years may very well merit
consideration of mass screening programs. However, the
prevalence of delayed-onset preschool hearing loss is only
one of several criteria to consider in the process of
justifying a population-based preschool screening
program.
Screening during preschool years is complicated by the
incidence of occult middle ear effusions associated with
minor upper respiratory illness. Such asymptomatic
effusions commonly resolve spontaneously and typically
require no intervention, but will complicate the screening
process as false positive test results requiring follow-up.
False positive rates in preschool screening programs have
been reported as high as 6–10% or more, in part due to
these asymptomatic middle ear effusions, and significantly
greater than the 1–4% false positive rates of most
hospital-based newborn screening programs (Hall, 2016).
Also contributing to the false positive rate is the challenge
of securing the cooperation of the preschool-aged child
to complete physiologic (OAE) testing. If the ideal age for
proposed universal preschool screening is in the area of
18–24 months, this same age would also be
acknowledged to be a time when young children are
typically the least cooperative with medical procedures
or testing. Additionally, false negative results with OAE
31
screening programs are a continuing concern. Due to the
limitations of hearing screening with this technology, OAE
testing may not identify children with mildly elevated
hearing levels 25 to 40 dB HL (Johnson, 2005a).
The feasibility of providing universal preschool-aged
hearing screening is also an important limiting factor.
UNHS has been a successful initiative in part because
almost all infants are born in a hospital, and hospital-based
programs therefore have the potential of capturing 95
percent or more of all newborns in a screening program
(Winston-Gerson & Hoffman, 2017). Similarly, the great
majority of children enter a public school system around
age five, and therefore school-based programs have
demonstrated similar successes at screening the hearing
of an entire population. However, during preschool years
there is no similar common door through which almost all
of the children pass.
The physician’s office is a setting that potentially would
capture most preschool-aged children for a mass
screening program (at an age range to be determined), but
not all children receive medical care in a timely way, due
to a multiplicity of factors related to families and systems
of care (Oberg, Colianni, & King-Schultz, 2016). Rather
than screening in the medical office, hearing screening
in pre-school facilities or through home visiting programs
could be an alternative mechanism, and such programs
have been demonstrated with Head Start and through
other preschool hearing screening initiatives (Council on
Community Pediatrics, 2009). Clearly, a significant portion
of the population of preschoolers in the United States may
be enrolled, at least at intervals, in larger daycare or
preschool settings. However, the proportion of such
enrolled children in the overall preschool population would
be far less than the 95% of newborns who are born in a
hospital, and so the success of a proposed universal
hearing screening program in this setting would be limited.
While acknowledging the concern about delayed-
onset hearing loss presenting during preschool years, the
JCIH finds that there is not adequate data to presently
justify a broader recommendation for universal hearing
screening during the preschool years. Further research
and technologic advances may allow for an expanded
recommendation in the future. Continued surveillance of
language development by the family, caretakers, and the
primary care provider, as well as observations of the child’s
responsiveness to auditory stimuli, is essential for
recognition and timely diagnosis of delayed-onset hearing
loss during preschool years.
Protecting the Rights of Infants/Toddlers and
Families
Every stakeholder involved in the EHDI process shares
responsibility for protecting rights and preserving
confidentiality. Families should receive information about
children who are deaf or hard of hearing and the potential
benefits and risks of proposed interventions. The
information should be presented in an easily-understood
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
manner and whenever possible, in the family’s preferred
language. It is important that families have the right to
accept or decline hearing screening tests or any follow-up
care for their newborn infant within statutory regulations,
just as they have such rights for any other screening or
evaluation procedures or intervention.
Additionally, all EHDI data merit the same level of
confidentiality and security afforded other health care and
education information in practice and law. The infant’s
family has the right to confidentiality of the screening
results and the follow-up assessments.
Three primary privacy regulations pertain to the exchange
of EHDI information:
• Health Insurance Portability and Accountability Act
(HIPAA) (U.S. Department of Health and Human
Services, 2017)
• Family Educational Rights and Privacy Act
(FERPA; U.S. Department of Education, n.d.-b)
• Part C regulations of IDEA (2004; U.S. Department of
Education, n.d.-a; NCHAM, 2013)
The HHS Office for Civil Rights has responsibility for
enforcing HIPAA rules with voluntary compliance activities
and civil monetary penalties (U.S. Department of Health
and Human Services, 2017). HIPAA regulations include
both privacy and security rules. The Standards for
Privacy of Individually Identifiable Health Information
(Privacy Rule) establishes national standards for protected
health information (PHI) in any medium (electronic, written,
or oral) and establishes patient rights with respect to that
information. The Privacy Rule permits PHI disclosure for
necessary patient care and disclosure to public health and
safety authorities. HIPAA regulations permit the sharing
of health information among health care professionals,
and the language in these regulations should facilitate the
prompt audiologic and medical evaluation of an infant who
does not pass the initial hearing screening tests.
Furthermore, the HIPAA Security Standards for the
Protection of Electronic Protected Health Information
(Security Rule) provides national security standards that
must be put in place to secure individuals’ electronic
protected health information (e-PHI). The Security Rule
specifies a series of additional administrative, physical,
and electronic security practice safeguards to ensure the
confidentiality, integrity, and availability of e-PHI,
regardless of how they are delivered or accessed,
including over the Internet. Under the HIPAA Security Rule,
health care providers, hospitals, and clinics are required
to implement policies and procedures to prevent, detect,
contain, and correct security violations.
The NCHAM has several resources addressing the impact
of privacy regulations (NCHAM, 2013) including a white
paper “How EHDI, Part C, and Health Providers can
Ensure that Children and Families Get Needed Services”
(NCHAM, 2008). As noted in the report, strategies can be
implemented to comply with signed consent requirement of
Part C privacy regulations (which are more restrictive than
FERPA) for the exchange of EHDI information.
32
The recommendations of the workgroup who contributed
to the report included:
• Provision of copies of diagnostic evaluation results,
treatment plans, IFSPs, and any signed consent forms
to families;
• Development of coordinated consent forms
incorporating the elements required by HIPAA, FERPA,
and Part C Privacy Regulations;
• Development of Memoranda of Agreement (MOA’s)
among EHDI, Part C, and Family Support Programs;
• Obtaining written consent from the family when
newborns do not pass hearing screening or as part of
the IFSP/IEP process;
• Development of state administrative rules and
regulations or laws mandating the reporting of EHDI
information to state programs and to the infant’s
medical home.
EHDI Information Technology Infrastructure
According to the Institute of Medicine (IOM), computerized
clinical data and decision support systems are a
prerequisite for the safe and comprehensive provision of
quality care (Institute of Medicine, 2001). The IOM
definition of quality is: “The degree to which health
services for individuals and populations increase the
likelihood of desired health outcomes and are consistent
with current professional knowledge” (p. 232).
Overall, there have been improvements in the provision
and documentation of screening and follow-up services in
the United States, which have resulted in the early
identification of more deaf and hard of hearing infants. This
progress has been possible through improvements in the
functionality of EHDI information systems and increases in
the ability of EHDI programs to successfully track infants
in need of follow-up services. However, some challenges
remain in ensuring the receipt of follow-up services and
additional efforts are needed to ensure all deaf and hard of
hearing infants are identified early and receive
intervention. These challenges include:
• Variation in the consistent reporting of follow-up data
across EHDI programs, which impacts the
completeness and quality of data;
• Differences in the infrastructure and capabilities of
EHDI information systems, which limits the ability of
some programs to accurately identify, match, collect,
and report data on all births that is unduplicated and
individually identifiable;
• Adoption of standard data definitions and varied
performance measures among EHDI programs,
potentially resulting in some differences in reported
results;
• Understanding of data reporting requirements among
providers and the burden to report data;
• Variation in the EHDI programs to measure and
improve quality through continuous feedback and the
application of computerized decision support; and
• Reliance to a great extent on 20th century
measurement technology rather than 21st century
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
technology (e.g., abstraction of samples of medical
records and the reliance upon information from
administrative datasets).
The JCIH promotes an information technology
infrastructure and national quality enhancement strategy
focused on performance measurement of clinical
quality and family satisfaction. The JCIH encourages
further development, refinement, and pilot testing of
well-defined EHDI performance measures, promotes the
adoption and enhancement of EHDI information system
infrastructure for comparing performance and
disseminating results, and recommends a research
agenda to support quality enhancement. The JCIH
recognizes the need to address the issues most important
to the provision of services and knowledge generation,
while ensuring security safeguards for data, adequately
protecting family privacy and limiting the burden of health
data collection.
EHDI programs, whenever possible, should prepare for
full implementation and adoption of nationally recognized
standard data definitions and standardized measures to
facilitate information exchange and analysis (Gaffney,
Eichwald, Gaffney, & Alam, 2014). In further refining
their EHDI information systems, stakeholders should not
reinvent the wheel, but rather build on and leverage work
already underway in both the private and public sectors to
establish a common conceptual framework for terminology
definitions and standardized quality measures. In
particular, electronic health data exchange standards for
recording and transmitting newborn screening test results
developed by the U. S. National Library of Medicine and
child health quality measures endorsed by the National
Quality Forum (NQF) should be adopted at the earliest
possible time (CDC, n.d.-b; NQF, n.d.-a, n.d.-b, n.d.-c,
n.d.-d). Resources for Early Hearing Detection and
Intervention and Electronic Health Records Technology are
available at the CDC website (CDC, n.d.-b).
The JCIH recognizes the need to bolster the capacity and
capabilities of EHDI programs for information exchange,
ensuring that data collected in one system can be used by
other systems for a variety of different uses (e.g.,
provision of services, quality assurance, research, and
public health). Much of EHDI information exchange
currently relies on paper forms that are mailed, emailed, or
faxed, necessitating manual data entry and coding by the
public health agency prior to initiating follow-up services,
analysis, or reporting. The JCIH encourages programs
and providers to migrate from paper-based health record
systems to an information infrastructure that captures and
stores data electronically and takes advantage of
computer-aided decision support.
A functional foundation for an EHDI information system
should have the ability to electronically collect, rather than
manually enter, screening results and demographic
information; accept, create, and report both clinical
decision support and quality measures; and, leverage
evolving local, regional, and national Health Information
33
Exchange (HIE) capabilities. An EHDI information
technology infrastructure needs to incorporate all aspects
of quality enhancement, including the use of standardized
measures and appropriate sharing of data where doing so
will likely result in greater gains in the quality of services
and reduce the burden on providers and families.
Benchmarks and Quality Indicators
The JCIH supports the concept of regular measurements
of performance and recommends routine monitoring of
these measures for inter-program comparison and
continuous quality improvement. These performance
benchmarks represent a consensus of expert opinion in
the field of newborn hearing screening and intervention.
The benchmarks are the minimal requirements that should
be attained by high-quality EHDI programs. Frequent
measures of quality permit prompt recognition and
correction of any unstable component of the EHDI
process.
Quality Measurement and Improvement
The provision of EHDI services can be improved and
better coordinated when data are captured to measure
performance and that information is shared among all
stakeholders. Use of consensus-based standardized
measures lessens reporting burden, focuses on a discrete
targeted set of measures to improve services, and allows
stakeholders to compare results.
To report and ensure information is accurate, complete,
and transparent, all measures should have clear,
unambiguous definitions for each numerator and
denominator with well-defined exclusions/exceptions and
data elements/value sets used for calculation. Whenever
possible, nationally endorsed measures and standard data
elements/coded value sets should be used. In addition,
steps should be taken to measure and report
individual-level geographic and demographic data.
National standard EHDI data elements/value sets are
maintained and available for public use through:
• Agency for Healthcare Research and Quality (AHRQ,
n.d.) U. S. Health Information Knowledge Base (USHIK)
• CDC (n.d.-c) Public Health Information Network (PHIN)
Vocabulary Access and Distribution System (VADS)
• National Library of Medicine (NLM) Newborn Screening
Coding and Terminology Guide (NLM, n.d.-a)
• NLM Value Set Authority Center (VSAC; NLM, n.d.-b)
EHDI has three measures endorsed by the NQF:
• Hearing screening prior to hospital discharge
(NFQ1354) [NQF, n.d.-b]
• Audiological evaluation no later than 3 months of age
(NFQ1360; NQF, n.d.-a)
• Intervention no later than 6 months of age (NFQ1361;
NQF, n.d.-c]
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
These NQF measures were developed and are stewarded
by CDC through a formal process for updating and
maintenance. Additional EHDI quality measures need to
ensure a rigorous and consensus-based process of
development involving all stakeholders. To ensure
accountability, individual, community, and state health and
educational programs should assume the responsibility for
coordinated, ongoing measurement and improvement of
EHDI processes and developmental outcomes. As
relevant quality measures are developed and
communicated, stakeholder organizations should address
what is important to achieve the best outcomes for deaf
and hard-of-hearing children and their families, without
creating an undue burden of data collection. Rather than
promoting specific recommended benchmarks, JCIH
strongly encourages the documentation of current baseline
measurements and establishment of quality improvement
activities for documenting continuous and measurable
improvements in screening, confirmation of hearing status,
and receipt of intervention services.
Quality indicators for screening. Quality indicators for
newborn hearing screening are:
• Percentage of all newborn infants who complete
screening by one month of age;
• Percentage of all newborn infants who do not pass
initial hospital-based screening and require subsequent
outpatient rescreening;
• Percentage of newborn infants who do not pass initial
and any/all subsequent rescreening(s) prior to
comprehensive audiologic evaluation; and
• Percentage of newborn infants who do not pass initial
screening and subsequently pass a re-screening.
Quality indicators for confirmation that a child is deaf
or hard of hearing. Quality indicators for confirmation of
hearing status and diagnosis of hearing thresholds are:
• Percentage of infants who do not pass initial birth
screening and any subsequent rescreening, and
• Percentage of infants who complete a comprehensive
audiologic evaluation by three months of age.
For families who elect amplification:
• Percentage of deaf and hard of hearing infants
receiving amplification devices within one month of
confirmation of hearing status.
Quality indicators for early intervention. Quality
indicators for early intervention for infants confirmed as
deaf or hard of hearing and qualify for Part C services
include:
• Percentage of infants for whom parents have signed an
IFSP no later than six months of age.
For children who are deaf or hard of hearing and have
experienced late-identification or delayed-onset
progression in hearing thresholds:
34
 • Percentage for whom parents have signed an
individualized family service plan within forty-five days
of the diagnosis.
• For all infants confirmed as deaf or hard of hearing:
• Percentage of infants who receive the first
developmental assessment using standardized
assessment protocols (not criterion reference
checklists) in the language of the home for spoken and/
or visual language, speech, and nonverbal cognitive
development no later than twelve months of age.
Effective January 1, 2016, The Joint Commission (TJC),
formerly known as the Joint Commission on
Accreditation of Healthcare Organizations (JCAHO),
adopted the EHDI electronic Clinical Quality Measure
(eCQM) for data reporting of newborn hearing screening
before hospital discharge (Eichwald, 2016). Any
accredited hospital may choose this measure as one of the
six required sets to satisfy their accreditation and
certification process. The 2016 Flexible Reporting Options
can be accessed from TJC’s website posted under the
“Measurement” section, ORYX Performance Measurement
Reporting (Joint Commission, n.d.). The Joint Commission
accreditation is recognized by a majority of state
governments in lieu of a hospital licensure inspection
conducted by the state-licensing agency and by the
Centers for Medicare and Medicaid Services in lieu of a
Medicare certification survey, thus qualifying organizations
to receive Medicare and Medicaid reimbursement.
Future Directions
In addition to the Benchmarks and Quality Indicators, best
practices in the diagnosis and management of infants and
children who are deaf or hard of hearing will continue to
evolve in myriad ways, impacted by multiple factors.
Future directions and goals for the EHDI process include
the following points.
Screening
• Better definition of hearing levels that are identified,
and missed, by current hearing screening technologies
to define sensitivity and specificity of both AABR and
OAE;
• Individualized in-ear calibration of the screening
stimulus to improve sensitivity and specificity; and
• Consideration of universal preschool hearing
screening, including cost and prognosis for improved
outcomes.
Diagnosis
• Continued development of new technologies and
stimuli to speed diagnosis and improve accuracy;
• Development of calibration standards for atypical
stimuli used in infant diagnosis for all transducers,
permitting better estimates of type and degree of
hearing loss; and
• Continued increase in the number of audiologists with
pediatric knowledge and skills with further reduction in
the serious shortage of qualified providers.
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
Amplification and Cochlear Implant Protocols and
Technologies
• Continued refinement in amplification, verification, and
validation protocols to facilitate access to speech;
• Increased systems initiatives from state and local EHDI
partners to support timely access and reduce barriers
to hearing aid amplification;
• Increased monitoring of hearing aid usage through
automated software programs that permit targeted
parent-counseling;
• Increased number of children who are fit with hearing
aid technologies, particularly those employing on-board
wireless capabilities, designed to benefit all children
who are fitted with amplification;
• Development of policies and procedures that result in
increases in state-funding or third-party payers for
amplification and other technologies;
• Improvement of protocols (preferably automated) and
additional development of technologies to assess
speech discrimination in children of all ages using
physiological cortical auditory evoked potentials and
behavioral procedures;
• Adherence to best practice evidence-based protocols
for fitting pediatric-specific hearing aids to children;
• Expansion of criteria for cochlear implantation including
lowered age of candidacy, as well as other options
(unilateral hearing loss, hybrid-devices for children with
substantial residual hearing) to provide earlier auditory
access to a larger population of children.
Healthcare Reform and Data Management Systems
• Evidence supported by healthcare reform, improved
data-management systems and new laws/regulations
to better define incidence and prevalence of all types of
hearing loss;
• Improved methods of data-collection, transmission, and
data analytics positively impacting the ability to observe
trends and modify diagnostic and intervention
strategies accordingly; and
• Focused efforts to increase long-term data of children
identified through EHDI programs offering insights into
gaps in services, equitable access and efficacy.
Tele-Practice
• Provision of services through innovative methods such
as telehealth resulting in more timely access and
reduction of barriers that impact outcomes;
• Promotion of evidence-based policy changes needed
to provide assurance of reimbursement for tele-practice
services; and
• Expansion of tele-practice, as a constantly evolving
mechanism for using technology to deliver a wide range
of professional EHDI services.
Several issues need to be addressed before the benefits
of tele-practice may be realized on a wide-scale basis.
These issues include the need for the following elements.
• Development of professional preparation and ongoing
training to maintain expertise and familiarity with
changes in technology and potential clinical
applications (see ASHA practice portal on tele-practice);
35
 • Establishment of state licensure requirements and
monitoring for tele-practice competencies for
practitioners;
• Exploration of and advocacy for additional funding
sources willing to reimburse providers for services
rendered; and
• Identification of pre-requisite family skills needed to
benefit from tele-practice delivery of services (e.g.,
able to navigate a computer, access technology,
computer literate, etc.).
Medical
• Emergence of additional genetic discoveries and other
medical breakthroughs to inform our understanding of
congenital and late-onset or progressive hearing loss;
• Assurance of access to genetic testing for all families
fostering an expansion in our ability to determine
etiology while increasing our understanding of the
underpinnings of congenital hearing loss;
• Recognition that while promising, the feasibility of wide
scale implementation of gene-based therapies are
unlikely in the near future due to heterogeneity of the
etiology of hearing loss as well as cultural preferences;
• Consideration and ongoing investigation of screening
for cCMV as a standard of care in all infants who do not
pass their newborn hearing screen;
• Development of targeted interventions for children who
are deaf or hard of hearing with additional disabilities in
an effort to reduce the impact of comorbidities;
• Advances in hearing preservation surgery and cochlear
implant candidacy have enormous potential for
impacting the timing of this intervention;
• Reliable hearing preservation surgery will make
cochlear implantation an earlier option for children
following diagnosis; and
• Potential candidacy for early implant technology for
unilateral/asymmetric hearing loss in select instances.
Early Intervention
In addition to this current 2019 position statement, early
intervention professionals are encouraged to continue
referring to the JCIH Supplement 2013. That document
provided extensive coverage of best practice principles to
guide interventions; only selected concepts are reinforced
here.
• Increased timely support and guidance for families of
newly identified infants to ensure that children have
access to high quality language input (regardless of
modalities or languages) from the earliest possible age;
• Provision of accurate and up-to-date information to
families about all language and communication
opportunities for their child, including visual, tactile, and
listening technologies that support language learning;
• Participation in professional development and training
to meet the changing nature and needs of families,
preparing professionals with the knowledge and skills to
provide services that are culturally and linguistically
sensitive and responsive in all respects;
• Recognition of the increasing number of culturally
and linguistically diverse children who are deaf or hard
The Journal of Early Hearing Detection and Intervention 2019; 4(2)
of hearing in the United States and its territories with
targeted efforts to increase cultural competence;
• Intentional outreach and diversification of the workforce
through both direct recruitment and increased access
for underrepresented groups to professional
preparation programs at universities so that families will
have an increased likelihood of working with a
professional of the same race or culture;
• Increased access to and inclusion of the Deaf
Community as an important resource for families and
providers in an effort to provide well-rounded services
for families;
• Development of longitudinal research agendas to guide
differentiation of practice and to expand evidence
regarding specific interventions and intervention
components and to inform family decision-making;
• Seamless access to and connections with family
service coordinators with specialized knowledge of the
needs of families and children who are deaf or hard of
hearing, thereby decreasing time to service initiation;
• Development of strategies to connect families with
resources and professionals providing adjustment
counseling services (psychologists, counselors, infant
mental health specialists) familiar with serving families
of children who are deaf and hard of hearing;
• Increased progress toward integration of EHDI
programs posited within state and territory agencies
charged with implementation of Part B and Part C IDEA
legislation;
• Collaboration with community evidence-based home
visiting programs supported by rigorous research and
recognized by the Maternal and Child Health Bureau to
provide families with resources that may supplement
early intervention programs;
• Broadening access to professionals with the skills, and
knowledge, and disposition to deliver high quality early
intervention services despite geographic, financial, and
other barriers through technology (i.e., tele-practice).
Research Needs
• Continued and accelerated research into
optimizing screening, diagnostic and amplification
intervention protocols, emphasizing timeliness and
accuracy based on rigorous evidence regarding
efficacy;
• Exploration of pre-school hearing screening programs
to determine the ability to identify late-onset or missed
hearing loss;
• Increased longitudinal research on the efficacy and
quality of early intervention strategies to assure optimal
outcomes (developmental and quality of life) for
children who are deaf or hard of hearing and their
families;
• Increased inquiry and study of the cost utility/benefit of
EHDI programs and systems;
• Focused research on the impact of social determinants
of health outcomes relative to deafness or hearing loss;
and
36
 • Further examination and delineation of health
disparities due to geographic location (rural, inner-city),
socio-economics, ethnic/racial groups and education.
Acknowledgments
The Year 2019 Position Statement was co-authored by the
members of the Joint Committee on Infant Hearing (JCIH).
The JCIH is funded by annual dues from each member
organization to cover operational costs (e.g., member
travel to meetings, sign language interpreters). All
contributors conduct the work as volunteers on behalf
of the organizations they represent and the JCIH. They
receive no remuneration and they have no conflicts of
interest to declare. The views and opinions expressed in
this position statement are those of the authors and do
not necessarily reflect the offical policy or position of the
member organizations.
JCIH member organizations and their respective
representatives who prepared this statement include (in
alphabetical order): the Alexander Graham Bell
Association for the Deaf and Hard of Hearing (Donald
Goldberg, PhD, and Carianne Muse, MPH); the
American Academy of Audiology (Alison M. Grimes, AuD,
and Christine Yoshinaga-Itano, PhD); the American
Academy of Otolaryngology–Head and Neck Surgery
(Craig Buchman, MD, and Oliver Adunka, MD); the
American Academy of Pediatrics (Rachel St. John, MD,
and Betty Vohr, MD); the American Speech-
Language-Hearing Association (Patti Martin, PhD, and
Ryan McCreery, PhD); the Council on Education of the
Deaf, whose member organizations include the Alexander
Graham Bell Association for the Deaf and Hard of Hearing,
the American Society for Deaf Children, the Association
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